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Thrown From Motorcycle
ANSWER
Several findings are evident from this radiograph. First, the quality is slightly diminished due to the patient’s size and artifact from the backboard. The patient’s mediastinum is somewhat widened, which is concerning for possible occult chest/vascular injury. There is some haziness within the left apical region suggestive of a hemothorax; no definite pneumothorax is seen. The left clavicle is fractured and displaced, and the left scapula is fractured as well.
ANSWER
Several findings are evident from this radiograph. First, the quality is slightly diminished due to the patient’s size and artifact from the backboard. The patient’s mediastinum is somewhat widened, which is concerning for possible occult chest/vascular injury. There is some haziness within the left apical region suggestive of a hemothorax; no definite pneumothorax is seen. The left clavicle is fractured and displaced, and the left scapula is fractured as well.
ANSWER
Several findings are evident from this radiograph. First, the quality is slightly diminished due to the patient’s size and artifact from the backboard. The patient’s mediastinum is somewhat widened, which is concerning for possible occult chest/vascular injury. There is some haziness within the left apical region suggestive of a hemothorax; no definite pneumothorax is seen. The left clavicle is fractured and displaced, and the left scapula is fractured as well.
A 57-year-old man is brought to your facility as a trauma code. He was riding a motorcycle on the highway, traveling approximately 45 to 50 mph, when the car in front of him abruptly stopped. He hit the car and was thrown from his bike. He believes he briefly lost consciousness but recalls emergency personnel tending to him. On arrival, he is awake and alert, complaining of pain in his neck, left arm, and left lower leg. Medical history is significant for borderline hypertension and a previous accident that resulted in an emergency laparotomy. Primary survey reveals stable vital signs: blood pressure of 157/100 mm Hg; heart rate, 110 beats/min; respiratory rate, 20 breaths/min; and O2 saturation, 98% with supplemental oxygen. Pupils are equal and reactive; there are slightly decreased breath sounds on the left side. Abdominal exam appears benign. There is decreased mobility and pain in the patient’s left upper and left lower extremities, although no obvious deformity is noted. Preliminary chest radiograph is obtained before the patient is sent for CT. What is your impression?
How Are Lesions and Seizures Related?
ANSWER
The correct answer is tuberous sclerosis (choice “d”), a genetic disorder affecting cellular differentiation and proliferation. As a result, hamartomas commonly form in organs such as the brain.
Von Recklinghausen disease (choice “a”; also known as neurofibromatosis type 1) presents with hyperpigmented macules and patches called café au lait macules. These and other findings differentiate it considerably from tuberous sclerosis.
“No unifying explanation” (choice “b”) is incorrect, because, as stated above, there is a unifying explanation for these findings!
Vitiligo (choice “c”) involves hypopigmented macules and patches. However, it does not present with some of the other findings seen in this case.
DISCUSSION
Tuberous sclerosis was first described in 1862 by von Recklinghausen, who noted the collection of findings we now term tuberous sclerosis complex (TSC) due to the wide variation in presentation.
In the United States, TSC occurs in an estimated one in 6,000 to one in 30,000 newborns. Usually, it manifests when the child is between ages 2 and 6, but genetic penetrance is highly variable—a major reason for the shift to the use of the term complex with this condition. (This case, with its late presentation, illustrates this variability.)
In about 60% of cases, TSC is transmitted in autosomal dominant mode, although at least 20% of cases may be due to spontaneous mutations. It has not shown any racial or gender predilections. Up to 90% of TSC patients will present with seizures, and 60% to 70% of those affected will demonstrate some developmental disability.
Skin is affected in at least 70% of TSC cases. This patient typifies the common dermatologic findings: dart-shaped hypopigmented macules (known as mountain ash leaf spots) and fleshy facial papules in the nasolabial region (originally called adenoma sebaceum and now called angiofibromas). Periungal fibromas are also common. Other major diagnostic criteria for TSC include cortical tubers seen on imaging and retinal hamartomas. Minor diagnostic criteria include pits in dental enamel and gingival fibromas.
Definitive diagnosis depends on identification of either two major manifestations or one major and two minor findings. There are no blood tests to confirm the diagnosis. In terms of treatment, the various elements that comprise TSC can be dealt with (eg, control of seizures and destruction or modification of skin lesions).
This patient received a diagnosis of TSC. She was returned to her primary care provider, who referred her for additional imaging of her lungs and heart and for ophthalmologic evaluation of her retinas. Genetic counseling was also arranged. With her seizures under control, she was able to continue matriculation through high school.
ANSWER
The correct answer is tuberous sclerosis (choice “d”), a genetic disorder affecting cellular differentiation and proliferation. As a result, hamartomas commonly form in organs such as the brain.
Von Recklinghausen disease (choice “a”; also known as neurofibromatosis type 1) presents with hyperpigmented macules and patches called café au lait macules. These and other findings differentiate it considerably from tuberous sclerosis.
“No unifying explanation” (choice “b”) is incorrect, because, as stated above, there is a unifying explanation for these findings!
Vitiligo (choice “c”) involves hypopigmented macules and patches. However, it does not present with some of the other findings seen in this case.
DISCUSSION
Tuberous sclerosis was first described in 1862 by von Recklinghausen, who noted the collection of findings we now term tuberous sclerosis complex (TSC) due to the wide variation in presentation.
In the United States, TSC occurs in an estimated one in 6,000 to one in 30,000 newborns. Usually, it manifests when the child is between ages 2 and 6, but genetic penetrance is highly variable—a major reason for the shift to the use of the term complex with this condition. (This case, with its late presentation, illustrates this variability.)
In about 60% of cases, TSC is transmitted in autosomal dominant mode, although at least 20% of cases may be due to spontaneous mutations. It has not shown any racial or gender predilections. Up to 90% of TSC patients will present with seizures, and 60% to 70% of those affected will demonstrate some developmental disability.
Skin is affected in at least 70% of TSC cases. This patient typifies the common dermatologic findings: dart-shaped hypopigmented macules (known as mountain ash leaf spots) and fleshy facial papules in the nasolabial region (originally called adenoma sebaceum and now called angiofibromas). Periungal fibromas are also common. Other major diagnostic criteria for TSC include cortical tubers seen on imaging and retinal hamartomas. Minor diagnostic criteria include pits in dental enamel and gingival fibromas.
Definitive diagnosis depends on identification of either two major manifestations or one major and two minor findings. There are no blood tests to confirm the diagnosis. In terms of treatment, the various elements that comprise TSC can be dealt with (eg, control of seizures and destruction or modification of skin lesions).
This patient received a diagnosis of TSC. She was returned to her primary care provider, who referred her for additional imaging of her lungs and heart and for ophthalmologic evaluation of her retinas. Genetic counseling was also arranged. With her seizures under control, she was able to continue matriculation through high school.
ANSWER
The correct answer is tuberous sclerosis (choice “d”), a genetic disorder affecting cellular differentiation and proliferation. As a result, hamartomas commonly form in organs such as the brain.
Von Recklinghausen disease (choice “a”; also known as neurofibromatosis type 1) presents with hyperpigmented macules and patches called café au lait macules. These and other findings differentiate it considerably from tuberous sclerosis.
“No unifying explanation” (choice “b”) is incorrect, because, as stated above, there is a unifying explanation for these findings!
Vitiligo (choice “c”) involves hypopigmented macules and patches. However, it does not present with some of the other findings seen in this case.
DISCUSSION
Tuberous sclerosis was first described in 1862 by von Recklinghausen, who noted the collection of findings we now term tuberous sclerosis complex (TSC) due to the wide variation in presentation.
In the United States, TSC occurs in an estimated one in 6,000 to one in 30,000 newborns. Usually, it manifests when the child is between ages 2 and 6, but genetic penetrance is highly variable—a major reason for the shift to the use of the term complex with this condition. (This case, with its late presentation, illustrates this variability.)
In about 60% of cases, TSC is transmitted in autosomal dominant mode, although at least 20% of cases may be due to spontaneous mutations. It has not shown any racial or gender predilections. Up to 90% of TSC patients will present with seizures, and 60% to 70% of those affected will demonstrate some developmental disability.
Skin is affected in at least 70% of TSC cases. This patient typifies the common dermatologic findings: dart-shaped hypopigmented macules (known as mountain ash leaf spots) and fleshy facial papules in the nasolabial region (originally called adenoma sebaceum and now called angiofibromas). Periungal fibromas are also common. Other major diagnostic criteria for TSC include cortical tubers seen on imaging and retinal hamartomas. Minor diagnostic criteria include pits in dental enamel and gingival fibromas.
Definitive diagnosis depends on identification of either two major manifestations or one major and two minor findings. There are no blood tests to confirm the diagnosis. In terms of treatment, the various elements that comprise TSC can be dealt with (eg, control of seizures and destruction or modification of skin lesions).
This patient received a diagnosis of TSC. She was returned to her primary care provider, who referred her for additional imaging of her lungs and heart and for ophthalmologic evaluation of her retinas. Genetic counseling was also arranged. With her seizures under control, she was able to continue matriculation through high school.
The parents of this 16-year-old girl first noticed the “spots” on their daughter’s thigh when she was 2. At that time, they expressed mild concern to the child’s pediatrician, who advised them to watch the lesions for change. In the absence of any, the spots were essentially ignored. But recently—and alarmingly—the patient experienced two grand mal seizures. Three months ago, she was hospitalized and underwent a thorough examination and workup, including imaging studies of the brain. These revealed presumed neural tumors, which are being followed with serial imaging. In the meantime, her primary care provider recommends a visit to dermatology for evaluation of the child’s skin—including the aforementioned spots on her thigh. Her parents deny any family history of similar problems with skin or seizures. The spots are located on the patient’s right lateral thigh. The largest is a dart-shaped 4 x 2.5–cm hypopigmented patch. It is surrounded by much smaller (< 1 cm in diameter) but similarly hypopigmented macules. The large lesion is symmetrical but has slightly serrated borders. Examination elsewhere reveals periungual fibromas on two of 10 fingers. Odd fleshy papules are noted in the bilateral nasolabial areas.
Man’s Heart Rhythm Has Been “Strange”
ANSWER
The ECG reveals sinus bradycardia with second-degree atrioventricular (AV) block (Mobitz I), also known as Wenckebach block.
Mobitz I heart block often occurs with reversible reasons of conduction block at the level of the AV node. While the P-P intervals remain constant, conduction fatigue within the AV node results in the P-R interval becoming progressively longer, until the AV node completely blocks conduction from the atria to the ventricles. The process then repeats itself in a pattern of P to QRS groups.
In this case, there are three P waves for every two QRS complexes, resulting in a 3:2 pattern. The PR interval is longest prior to the blocked QRS and shortest immediately after it. The diagnosis of sinus bradycardia results from a constant P-P interval of 58 beats/min.
Further questioning of the patient revealed that he had inadvertently doubled his dose of metoprolol. Correcting this resulted in the return of normal sinus rhythm.
ANSWER
The ECG reveals sinus bradycardia with second-degree atrioventricular (AV) block (Mobitz I), also known as Wenckebach block.
Mobitz I heart block often occurs with reversible reasons of conduction block at the level of the AV node. While the P-P intervals remain constant, conduction fatigue within the AV node results in the P-R interval becoming progressively longer, until the AV node completely blocks conduction from the atria to the ventricles. The process then repeats itself in a pattern of P to QRS groups.
In this case, there are three P waves for every two QRS complexes, resulting in a 3:2 pattern. The PR interval is longest prior to the blocked QRS and shortest immediately after it. The diagnosis of sinus bradycardia results from a constant P-P interval of 58 beats/min.
Further questioning of the patient revealed that he had inadvertently doubled his dose of metoprolol. Correcting this resulted in the return of normal sinus rhythm.
ANSWER
The ECG reveals sinus bradycardia with second-degree atrioventricular (AV) block (Mobitz I), also known as Wenckebach block.
Mobitz I heart block often occurs with reversible reasons of conduction block at the level of the AV node. While the P-P intervals remain constant, conduction fatigue within the AV node results in the P-R interval becoming progressively longer, until the AV node completely blocks conduction from the atria to the ventricles. The process then repeats itself in a pattern of P to QRS groups.
In this case, there are three P waves for every two QRS complexes, resulting in a 3:2 pattern. The PR interval is longest prior to the blocked QRS and shortest immediately after it. The diagnosis of sinus bradycardia results from a constant P-P interval of 58 beats/min.
Further questioning of the patient revealed that he had inadvertently doubled his dose of metoprolol. Correcting this resulted in the return of normal sinus rhythm.
A 74-year-old man presents to your outpatient clinic for a routine appointment. He’s been your patient for years, but you’ve had difficulty convincing him of the importance of taking his hypertension medications regularly. For the most part, he has been compliant; however, recently, with finances becoming tight toward the end of the month, he often takes his β-blocker and diuretic every other day in order to stretch his prescription before refilling it. His health has remained excellent since you last saw him a year ago. However, while performing a review of systems, you learn that his heart rhythm has been “funny” in the past two weeks. He states it hasn’t affected his ability to perform his daily activities, including farming, but it was just “strange.” He denies chest pain, shortness of breath, dizziness, syncope or near-syncope, and peripheral edema. He still manages his 450-acre farm, as he has for most of his adult life. Medical history includes hypertension but no angina, MI, or other cardiac disease. Surgical history is remarkable for a right inguinal hernia repair, an appendectomy, and a right hip replacement. His medications include furosemide, potassium chloride, and metoprolol. He has no known drug allergies and does not use recreational drugs or naturopathic herbs. The patient has been a widower for 12 years. His two sons live nearby and help him on his farm. Due to his religious affiliation, he has never used alcohol or tobacco. Review of systems is remarkable for palpitations and an occasional skipped beat. Vital signs include a blood pressure of 108/58 mm Hg; pulse, 50 beats/min and “irregular”; respiratory rate, 14 breaths/min-1; temperature, 98.4°F; and O2 saturation, 96% on room air. His weight is 176 lb and his height, 74 in. Physical exam reveals a pulse that is regularly irregular at a rate of 56 beats/min. There are no murmurs, rubs, or gallops. The neck veins are not distended, and there is no peripheral edema. His lungs are clear to auscultation, and the remainder of his physical exam is unchanged from his previous visit. Given the change in his heart rhythm since his previous visit, you order an ECG and note the following: a ventricular rate of 44 beats/min; PR interval, not measured; QRS duration, 106 ms; QT/QTc interval, 484/413 ms; P axis, 65°; R axis, 11°; and T axis, 6°. What is your interpretation of this ECG?
Tender Thumbnail Papule
The Diagnosis: Myxoid Cyst
Myxoid cysts, often called ganglion cysts or mucous cysts, are smooth translucent nodules that arise between the dorsal aspect of the distal interphalangeal joint and proximal nail fold.1 Lesions often have an associated distal depression or groove on the affected fingernail and typically appear on the middle and index fingers between the fourth and seventh decades of life.2,3 Acute lesions may present as tender nodules, whereas gradually developing lesions tend to be painless.3 Trauma to the distal interphalangeal joint, degenerative processes, and osteoarthritis may increase hyaluronic acid production and allow synovial fluid to escape the joint space, accumulating in the surrounding tissue. Subungual localization of a mucous cyst is rare and may be difficult to diagnose.4 The differential diagnosis includes benign and malignant tumors such as periungual fibroma, glomus tumor, basal cell carcinoma, squamous cell carcinoma, and amelanotic melanoma.5 Our patient declined treatment with cryotherapy or intralesional steroid injection and drainage. He was referred to the orthopedic surgery department for surgical removal of the cyst and imaging studies. Radiographs of the right thumb revealed severe osteoarthritis of the distal interphalangeal joint, demonstrating the association of digital mucous cysts.
1. Karrer S, Hohenleutner U, Szeimies RM, et al. Treatment of a digital mucous cyst with a carbon dioxide laser. Acta Derm Venereol. 1999;79:224-225.
2. Brown RE, Zook EG, Russell RC. Fingernail deformities secondary to ganglions of the distal interphalangeal joint (mucous cysts). Plast Reconstr Surg. 1991;87:718-725.
3. de Berker D, Goettman S, Baran R. Subungual myxoid cysts: clinical manifestations and response to therapy. J Am Acad Dermatol. 2002;46:394-398.
4. Lin YC, Wu TH, Scher RK. Nail changes and association of osteoarthritis in digital myxoid cyst. Dermatol Surg. 2008;34:364-369.
5. Kivanc-Altunay I, Kumbasar E, Gokdemir G. Unusual localization of multiple myxoid (mucous) cysts of toes. Dermatol Online J. 2004;10:23.
The Diagnosis: Myxoid Cyst
Myxoid cysts, often called ganglion cysts or mucous cysts, are smooth translucent nodules that arise between the dorsal aspect of the distal interphalangeal joint and proximal nail fold.1 Lesions often have an associated distal depression or groove on the affected fingernail and typically appear on the middle and index fingers between the fourth and seventh decades of life.2,3 Acute lesions may present as tender nodules, whereas gradually developing lesions tend to be painless.3 Trauma to the distal interphalangeal joint, degenerative processes, and osteoarthritis may increase hyaluronic acid production and allow synovial fluid to escape the joint space, accumulating in the surrounding tissue. Subungual localization of a mucous cyst is rare and may be difficult to diagnose.4 The differential diagnosis includes benign and malignant tumors such as periungual fibroma, glomus tumor, basal cell carcinoma, squamous cell carcinoma, and amelanotic melanoma.5 Our patient declined treatment with cryotherapy or intralesional steroid injection and drainage. He was referred to the orthopedic surgery department for surgical removal of the cyst and imaging studies. Radiographs of the right thumb revealed severe osteoarthritis of the distal interphalangeal joint, demonstrating the association of digital mucous cysts.
The Diagnosis: Myxoid Cyst
Myxoid cysts, often called ganglion cysts or mucous cysts, are smooth translucent nodules that arise between the dorsal aspect of the distal interphalangeal joint and proximal nail fold.1 Lesions often have an associated distal depression or groove on the affected fingernail and typically appear on the middle and index fingers between the fourth and seventh decades of life.2,3 Acute lesions may present as tender nodules, whereas gradually developing lesions tend to be painless.3 Trauma to the distal interphalangeal joint, degenerative processes, and osteoarthritis may increase hyaluronic acid production and allow synovial fluid to escape the joint space, accumulating in the surrounding tissue. Subungual localization of a mucous cyst is rare and may be difficult to diagnose.4 The differential diagnosis includes benign and malignant tumors such as periungual fibroma, glomus tumor, basal cell carcinoma, squamous cell carcinoma, and amelanotic melanoma.5 Our patient declined treatment with cryotherapy or intralesional steroid injection and drainage. He was referred to the orthopedic surgery department for surgical removal of the cyst and imaging studies. Radiographs of the right thumb revealed severe osteoarthritis of the distal interphalangeal joint, demonstrating the association of digital mucous cysts.
1. Karrer S, Hohenleutner U, Szeimies RM, et al. Treatment of a digital mucous cyst with a carbon dioxide laser. Acta Derm Venereol. 1999;79:224-225.
2. Brown RE, Zook EG, Russell RC. Fingernail deformities secondary to ganglions of the distal interphalangeal joint (mucous cysts). Plast Reconstr Surg. 1991;87:718-725.
3. de Berker D, Goettman S, Baran R. Subungual myxoid cysts: clinical manifestations and response to therapy. J Am Acad Dermatol. 2002;46:394-398.
4. Lin YC, Wu TH, Scher RK. Nail changes and association of osteoarthritis in digital myxoid cyst. Dermatol Surg. 2008;34:364-369.
5. Kivanc-Altunay I, Kumbasar E, Gokdemir G. Unusual localization of multiple myxoid (mucous) cysts of toes. Dermatol Online J. 2004;10:23.
1. Karrer S, Hohenleutner U, Szeimies RM, et al. Treatment of a digital mucous cyst with a carbon dioxide laser. Acta Derm Venereol. 1999;79:224-225.
2. Brown RE, Zook EG, Russell RC. Fingernail deformities secondary to ganglions of the distal interphalangeal joint (mucous cysts). Plast Reconstr Surg. 1991;87:718-725.
3. de Berker D, Goettman S, Baran R. Subungual myxoid cysts: clinical manifestations and response to therapy. J Am Acad Dermatol. 2002;46:394-398.
4. Lin YC, Wu TH, Scher RK. Nail changes and association of osteoarthritis in digital myxoid cyst. Dermatol Surg. 2008;34:364-369.
5. Kivanc-Altunay I, Kumbasar E, Gokdemir G. Unusual localization of multiple myxoid (mucous) cysts of toes. Dermatol Online J. 2004;10:23.
A 71-year-old man presented to the dermatology clinic with mild tenderness and disfigurement of the right thumbnail of 6 months’ duration. The patient reported trauma to his thumb from closing a window on it during the time between onset of symptoms and presentation to the dermatology clinic. On physical examination the right thumbnail was atrophic with a flesh-colored papule involving the proximal nail bed. The nail plate overlying the papule was thinned by the underlying growth and there was a linear groove extending from the papule to the end of the nail. A biopsy was recommended for diagnosis and lidocaine was injected into the proximal aspect of the nail fold for local anesthesia. The lidocaine filled the papule, resulting in increased subungual pressure that caused the lesion to rupture through the nail plate, extruding a clear mucoid substance.
Punctate Depigmented Macules
The Diagnosis: Blaschkoid Punctate Vitiligo
Based on the patient’s clinical appearance as well as the histologic findings, the diagnosis of vitiligo was made. Although vitiligo is certainly not uncommon and punctate vitiligo is a known clinical presentation,1 punctate vitiliginous depigmentation conforming to lines of Blaschko is unique. Follicular repigmentation in a patch of vitiligo potentially could lead to this “spotty” appearance, but our patient maintained that the band was never confluently depigmented and that small macules arose within normally pigmented skin. The patient’s adult age at onset makes this case even more unusual.
Follicular repigmentation in vitiligo is fairly well understood, as the perifollicular pigment is formed by upward migration of activated melanoblasts in the outer root sheath.2 Follicular depigmentation as well as selective or initial loss of melanocytes around hair follicles in early vitiligo has not been described. It is unclear if the seemingly folliculocentric nature of the patient’s vitiliginous macules was a false observation, coincidental, or actually related to selective melanocyte loss around follicles.
Blaschkoid distribution has been described in numerous skin disorders and is known to be based on genetic mosaicism.3 Most of these disorders are X-linked and/or congenital. However, many acquired skin conditions have been described exhibiting blaschkoid distribution, such as vitiligo, psoriasis, lichen planus, atopic dermatitis, and mycosis fungoides.4,5
Confettilike depigmentation has been described as an unusual clinical variant of vitiligo.1 It also has been reported after psoralen plus UVA therapy in patients with more classic vitiligo,6 numerous domestic chemicals,7 and in association with mycosis fungoides.8 In these cases, punctate lesions were disseminated, symmetric on extremities, or limited to areas exposed to chemicals.
1. Ortonne J-P. Vitiligo and other disorders of hypopigmentation. In: Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. Vol 1. 2nd ed. St. Louis, MO: Mosby; 2003:913-938.
2. Cui J, Shen LY, Wang GC. Role of hair follicles in the repigmentation of vitiligo. J Invest Dermatol. 1991;97:410-416.
3. Happle R. X-chromosome inactivation: role in skin disease expression. Acta Paediatr Suppl. 2006;95:16-23.
4. Taieb A. Linear atopic dermatitis (“naevus atopicus”): a pathogenetic clue? Br J Dermatol. 1994;131:134-135.
5. Bolognia JL, Orlow SJ, Glick SA. Lines of Blaschko. J Am Acad Dermatol. 1994;31:157-190.
6. Falabella R, Escobar CE, Carrascal E, et al. Leukoderma punctata. J Am Acad Dermatol. 1988;18:485-494.
7. Ghosh S, Mukhopadhyay S. Chemical leucoderma: a clinico-aetiological study of 864 cases in the perspective of a developing country. Br J Dermatol. 2009;160:40-47.
8. Loquai C, Metza D, Nashan D, et al. Confetti-like lesions with hyperkeratosis: a novel ultraviolet-induced hypomelanotic disorder? Br J Dermatol. 2005;153:190-193.
The Diagnosis: Blaschkoid Punctate Vitiligo
Based on the patient’s clinical appearance as well as the histologic findings, the diagnosis of vitiligo was made. Although vitiligo is certainly not uncommon and punctate vitiligo is a known clinical presentation,1 punctate vitiliginous depigmentation conforming to lines of Blaschko is unique. Follicular repigmentation in a patch of vitiligo potentially could lead to this “spotty” appearance, but our patient maintained that the band was never confluently depigmented and that small macules arose within normally pigmented skin. The patient’s adult age at onset makes this case even more unusual.
Follicular repigmentation in vitiligo is fairly well understood, as the perifollicular pigment is formed by upward migration of activated melanoblasts in the outer root sheath.2 Follicular depigmentation as well as selective or initial loss of melanocytes around hair follicles in early vitiligo has not been described. It is unclear if the seemingly folliculocentric nature of the patient’s vitiliginous macules was a false observation, coincidental, or actually related to selective melanocyte loss around follicles.
Blaschkoid distribution has been described in numerous skin disorders and is known to be based on genetic mosaicism.3 Most of these disorders are X-linked and/or congenital. However, many acquired skin conditions have been described exhibiting blaschkoid distribution, such as vitiligo, psoriasis, lichen planus, atopic dermatitis, and mycosis fungoides.4,5
Confettilike depigmentation has been described as an unusual clinical variant of vitiligo.1 It also has been reported after psoralen plus UVA therapy in patients with more classic vitiligo,6 numerous domestic chemicals,7 and in association with mycosis fungoides.8 In these cases, punctate lesions were disseminated, symmetric on extremities, or limited to areas exposed to chemicals.
The Diagnosis: Blaschkoid Punctate Vitiligo
Based on the patient’s clinical appearance as well as the histologic findings, the diagnosis of vitiligo was made. Although vitiligo is certainly not uncommon and punctate vitiligo is a known clinical presentation,1 punctate vitiliginous depigmentation conforming to lines of Blaschko is unique. Follicular repigmentation in a patch of vitiligo potentially could lead to this “spotty” appearance, but our patient maintained that the band was never confluently depigmented and that small macules arose within normally pigmented skin. The patient’s adult age at onset makes this case even more unusual.
Follicular repigmentation in vitiligo is fairly well understood, as the perifollicular pigment is formed by upward migration of activated melanoblasts in the outer root sheath.2 Follicular depigmentation as well as selective or initial loss of melanocytes around hair follicles in early vitiligo has not been described. It is unclear if the seemingly folliculocentric nature of the patient’s vitiliginous macules was a false observation, coincidental, or actually related to selective melanocyte loss around follicles.
Blaschkoid distribution has been described in numerous skin disorders and is known to be based on genetic mosaicism.3 Most of these disorders are X-linked and/or congenital. However, many acquired skin conditions have been described exhibiting blaschkoid distribution, such as vitiligo, psoriasis, lichen planus, atopic dermatitis, and mycosis fungoides.4,5
Confettilike depigmentation has been described as an unusual clinical variant of vitiligo.1 It also has been reported after psoralen plus UVA therapy in patients with more classic vitiligo,6 numerous domestic chemicals,7 and in association with mycosis fungoides.8 In these cases, punctate lesions were disseminated, symmetric on extremities, or limited to areas exposed to chemicals.
1. Ortonne J-P. Vitiligo and other disorders of hypopigmentation. In: Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. Vol 1. 2nd ed. St. Louis, MO: Mosby; 2003:913-938.
2. Cui J, Shen LY, Wang GC. Role of hair follicles in the repigmentation of vitiligo. J Invest Dermatol. 1991;97:410-416.
3. Happle R. X-chromosome inactivation: role in skin disease expression. Acta Paediatr Suppl. 2006;95:16-23.
4. Taieb A. Linear atopic dermatitis (“naevus atopicus”): a pathogenetic clue? Br J Dermatol. 1994;131:134-135.
5. Bolognia JL, Orlow SJ, Glick SA. Lines of Blaschko. J Am Acad Dermatol. 1994;31:157-190.
6. Falabella R, Escobar CE, Carrascal E, et al. Leukoderma punctata. J Am Acad Dermatol. 1988;18:485-494.
7. Ghosh S, Mukhopadhyay S. Chemical leucoderma: a clinico-aetiological study of 864 cases in the perspective of a developing country. Br J Dermatol. 2009;160:40-47.
8. Loquai C, Metza D, Nashan D, et al. Confetti-like lesions with hyperkeratosis: a novel ultraviolet-induced hypomelanotic disorder? Br J Dermatol. 2005;153:190-193.
1. Ortonne J-P. Vitiligo and other disorders of hypopigmentation. In: Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. Vol 1. 2nd ed. St. Louis, MO: Mosby; 2003:913-938.
2. Cui J, Shen LY, Wang GC. Role of hair follicles in the repigmentation of vitiligo. J Invest Dermatol. 1991;97:410-416.
3. Happle R. X-chromosome inactivation: role in skin disease expression. Acta Paediatr Suppl. 2006;95:16-23.
4. Taieb A. Linear atopic dermatitis (“naevus atopicus”): a pathogenetic clue? Br J Dermatol. 1994;131:134-135.
5. Bolognia JL, Orlow SJ, Glick SA. Lines of Blaschko. J Am Acad Dermatol. 1994;31:157-190.
6. Falabella R, Escobar CE, Carrascal E, et al. Leukoderma punctata. J Am Acad Dermatol. 1988;18:485-494.
7. Ghosh S, Mukhopadhyay S. Chemical leucoderma: a clinico-aetiological study of 864 cases in the perspective of a developing country. Br J Dermatol. 2009;160:40-47.
8. Loquai C, Metza D, Nashan D, et al. Confetti-like lesions with hyperkeratosis: a novel ultraviolet-induced hypomelanotic disorder? Br J Dermatol. 2005;153:190-193.
An otherwise healthy 54-year-old black man presented with a 10-year history of spotty pigmentary loss in a band on the left side of the abdomen, flank, and back. He denied a history of rash or inflammation in the area and had not experienced confluent depigmentation. He reported that initially he had only a few “white dots,” and over the next 5 to 7 years, he developed more of them confined within the same area. On presentation, he stated new areas of depigmentation had not developed in several years. The band was completely asymptomatic and had not been treated with any prescription or over-the-counter medications. On examination he had multiple 2- to 3-mm confettilike depigmented macules that seemed to be centered around follicles in a band with blaschkoid distribution extending across the left side of the abdomen, flank, and back. The band did not cross the midline and similar lesions were not present elsewhere. A punch biopsy of one of the depigmented macules revealed a markedly diminished number of melanocytes along the junction as well as a decrease in melanin, which was confirmed by Melan-A and Fontana stains, respectively.
Clipped by an Oncoming Car
ANSWER
The image shows a comminuted and depressed fracture of the lateral tibial plateau. It is depressed approximately 6 to 7 mm. The patient was admitted, and orthopedic consultation was obtained. The patient subsequently underwent an open reduction and internal fixation of the fracture.
ANSWER
The image shows a comminuted and depressed fracture of the lateral tibial plateau. It is depressed approximately 6 to 7 mm. The patient was admitted, and orthopedic consultation was obtained. The patient subsequently underwent an open reduction and internal fixation of the fracture.
ANSWER
The image shows a comminuted and depressed fracture of the lateral tibial plateau. It is depressed approximately 6 to 7 mm. The patient was admitted, and orthopedic consultation was obtained. The patient subsequently underwent an open reduction and internal fixation of the fracture.
A 23-year-old man is brought in after being hit by a car. He was in the process of getting into his car when another vehicle coming from the opposite direction swerved into his lane. He tried to jump onto his hood to avoid the other car but was struck by the side mirror and landed on the ground. He is primarily complaining of left knee and lower leg pain. He denies any medical history. Primary survey appears to be stable except for scalp and facial lacerations. The patient is awake, alert, and oriented, and his vital signs are stable. His left lower extremity is in a splint immobilizer, placed by emergency medical personnel. There is a moderate amount of soft tissue swelling around the knee, which is exquisitely tender to palpation. The patient has limited flexion and extension of the knee due to pain. He is able to wiggle his toes, and distally in the leg and foot there appears to be no neurovascular compromise. Radiographs of the tibia are obtained. What is your impression?
No Time for Chest Pain When There Are Chores to Do
ANSWER
There are three significant findings on this ECG. First, the rhythm shows complete heart block. The ventricular rate is 56 beats/min, and the QRS complex is narrow, resulting in a junctional rhythm. The atrial rate is 98 beats/min (consistent with a sinus rhythm), and there is no relationship of the P waves to the QRS complexes.
The second finding is a rightward axis deviation. Note that the QRS complexes are negative in lead I and positive in lead aVF. To meet criteria for a right-axis deviation, the QRS complex must also be positive in lead aVR. In this case, the QRS complex appears to be isoelectric in aVR, so we call the axis rightward.
The presence of rightward axis deviation is a result of the third finding, an anterior MI. This is due to the LAD artery occlusion discovered at catheterization. It is evident on the ECG by the absence of significant R waves in leads V1 through V4.
Given the need for a β-blocker with titration of dose, the patient underwent implantation of a permanent pacemaker system.
ANSWER
There are three significant findings on this ECG. First, the rhythm shows complete heart block. The ventricular rate is 56 beats/min, and the QRS complex is narrow, resulting in a junctional rhythm. The atrial rate is 98 beats/min (consistent with a sinus rhythm), and there is no relationship of the P waves to the QRS complexes.
The second finding is a rightward axis deviation. Note that the QRS complexes are negative in lead I and positive in lead aVF. To meet criteria for a right-axis deviation, the QRS complex must also be positive in lead aVR. In this case, the QRS complex appears to be isoelectric in aVR, so we call the axis rightward.
The presence of rightward axis deviation is a result of the third finding, an anterior MI. This is due to the LAD artery occlusion discovered at catheterization. It is evident on the ECG by the absence of significant R waves in leads V1 through V4.
Given the need for a β-blocker with titration of dose, the patient underwent implantation of a permanent pacemaker system.
ANSWER
There are three significant findings on this ECG. First, the rhythm shows complete heart block. The ventricular rate is 56 beats/min, and the QRS complex is narrow, resulting in a junctional rhythm. The atrial rate is 98 beats/min (consistent with a sinus rhythm), and there is no relationship of the P waves to the QRS complexes.
The second finding is a rightward axis deviation. Note that the QRS complexes are negative in lead I and positive in lead aVF. To meet criteria for a right-axis deviation, the QRS complex must also be positive in lead aVR. In this case, the QRS complex appears to be isoelectric in aVR, so we call the axis rightward.
The presence of rightward axis deviation is a result of the third finding, an anterior MI. This is due to the LAD artery occlusion discovered at catheterization. It is evident on the ECG by the absence of significant R waves in leads V1 through V4.
Given the need for a β-blocker with titration of dose, the patient underwent implantation of a permanent pacemaker system.
Three days ago, a 62-year-old man was admitted with chest pain and an MI (confirmed by cardiac enzymes). The chest pain started while he was working on his farm, but he did not seek immediate help because he assumed it was heartburn and he had chores to finish. When the pain did not resolve overnight, he finally presented to the emergency department. Cardiac catheterization revealed an occluded left anterior descending (LAD) artery distal to the first diagonal branch and diffuse disease in the circumflex and right coronary arteries. An echocardiogram showed diffuse left ventricular hypokinesis and no evidence of valvular disease. His left ventricular ejection fraction was estimated to be 48%. Medical history is remarkable for hypertension and gout. Surgical history is remarkable for an appendectomy at age 7 and surgical repair of a fractured tibia from a high school football injury. Family history is positive for coronary artery disease; both parents died at young ages (father at 60, mother at 65) of MI, and his older brother had an MI at age 50 (but is currently doing well). The patient owns a 475-acre farm on which he grows corn and soybeans. He also tends to 23 cows and has a large chicken coop. There are five workers to help, but he states that he does most of the work himself. He is divorced and lives alone with five dogs, whom he refers to as his “kids.” He does not smoke, but he has one beer and one shot of bourbon with dinner each night. His only medication at the time of admission was ibuprofen. He had been prescribed lisinopril in the past but hadn’t taken it in six months because “it’s too far a drive to get it refilled.” He has no known drug allergies. Following admission, he was started on a β-blocker (metoprolol), aspirin, atorvastatin, and lisinopril. During rounds, you notice that his hypertension is well controlled. His blood pressure is 118/80 mm Hg, compared to 180/92 mm Hg on admission. He is comfortable and wants to know when he can go home. As you contemplate discharge, a technician hands you the patient’s daily ECG tracing. It shows a ventricular rate of 56 beats/min; QRS duration, 106 ms; QT/QTc interval, 400/386 ms; P axis, 36°; R axis, 120°; and T axis, 7°. What is your interpretation of this ECG?
Man Is Alarmed by Skin Lesions
ANSWER
The correct answer is eruptive xanthomata (choice “b”) caused by an accumulation of lipid-filled macrophages as a result of pathologic levels of serum triglyceride—a situation discussed more fully below.
Neurofibromatosis type I (choice “a”), also known as von Recklinghausen disease, can present with multiple intradermal nodules. However, it usually appears in the second or third decade of life, with lesions that are fixed and soft. Biopsy would have confirmed this diagnosis.
Diabetic dermopathy (choice “c”) manifests with atrophic patches on anterior tibial skin. The patches occasionally become superficially eroded but do not resemble this patient’s lesions at all.
Juvenile xanthogranuloma (choice “d”) usually presents on children as a solitary yellowish brown papule. It can resemble eruptive xanthomata histologically but not clinically.
DISCUSSION
Eruptive xanthomata (EX) are relatively common, manifesting rapidly as papules and nodules, most frequently in the setting of hypertriglyceridemia. The latter can be familial and may be worsened by poorly controlled diabetes. Persons with Fredrickson types I, IV, and V hyperlipidemia are especially prone to EX.
As might be expected, patients with EX are at risk for several associated morbidities, including acute pancreatitis (especially in childhood cases) and atherosclerotic vessel disease. EX have also been associated with hypothyroid states and nephrotic syndromes.
Elevations in cholesterol, with normal triglyceride levels, can be associated with several types of xanthoma, including plane xanthomas and xanthelasma. The latter, often benign, can manifest in a normolipemic patient as well (necessitating a problem-directed history, physical, and lipid check).
Biopsy is often required to confirm the diagnosis of EX. As in this case, it typically shows monotonous collections of lipid-laden macrophages. Frozen sections of EX can be successfully stained for lipids, but routine processing of specimens effectively removes any lipids, replacing them with paraffin.
TREATMENT
Treatment entails controlling lipids with medication (fenofibrate), diet, and exercise and getting diabetes under control, as indicated. It is also essential to assess for atherosclerotic vessel disease and rule out pancreatitis.
Within a month of institution of treatment, this patient’s lesions had all but disappeared. His serum amylase and lipase were within normal limits, and testing for atherosclerotic vessel disease was pending.
Click here for more DermaDiagnosis cases, including
• The Value of Certainty in Diagnosis
• A Purplish Rash on the Instep
• Hair Loss at a Very Young Age.
ANSWER
The correct answer is eruptive xanthomata (choice “b”) caused by an accumulation of lipid-filled macrophages as a result of pathologic levels of serum triglyceride—a situation discussed more fully below.
Neurofibromatosis type I (choice “a”), also known as von Recklinghausen disease, can present with multiple intradermal nodules. However, it usually appears in the second or third decade of life, with lesions that are fixed and soft. Biopsy would have confirmed this diagnosis.
Diabetic dermopathy (choice “c”) manifests with atrophic patches on anterior tibial skin. The patches occasionally become superficially eroded but do not resemble this patient’s lesions at all.
Juvenile xanthogranuloma (choice “d”) usually presents on children as a solitary yellowish brown papule. It can resemble eruptive xanthomata histologically but not clinically.
DISCUSSION
Eruptive xanthomata (EX) are relatively common, manifesting rapidly as papules and nodules, most frequently in the setting of hypertriglyceridemia. The latter can be familial and may be worsened by poorly controlled diabetes. Persons with Fredrickson types I, IV, and V hyperlipidemia are especially prone to EX.
As might be expected, patients with EX are at risk for several associated morbidities, including acute pancreatitis (especially in childhood cases) and atherosclerotic vessel disease. EX have also been associated with hypothyroid states and nephrotic syndromes.
Elevations in cholesterol, with normal triglyceride levels, can be associated with several types of xanthoma, including plane xanthomas and xanthelasma. The latter, often benign, can manifest in a normolipemic patient as well (necessitating a problem-directed history, physical, and lipid check).
Biopsy is often required to confirm the diagnosis of EX. As in this case, it typically shows monotonous collections of lipid-laden macrophages. Frozen sections of EX can be successfully stained for lipids, but routine processing of specimens effectively removes any lipids, replacing them with paraffin.
TREATMENT
Treatment entails controlling lipids with medication (fenofibrate), diet, and exercise and getting diabetes under control, as indicated. It is also essential to assess for atherosclerotic vessel disease and rule out pancreatitis.
Within a month of institution of treatment, this patient’s lesions had all but disappeared. His serum amylase and lipase were within normal limits, and testing for atherosclerotic vessel disease was pending.
Click here for more DermaDiagnosis cases, including
• The Value of Certainty in Diagnosis
• A Purplish Rash on the Instep
• Hair Loss at a Very Young Age.
ANSWER
The correct answer is eruptive xanthomata (choice “b”) caused by an accumulation of lipid-filled macrophages as a result of pathologic levels of serum triglyceride—a situation discussed more fully below.
Neurofibromatosis type I (choice “a”), also known as von Recklinghausen disease, can present with multiple intradermal nodules. However, it usually appears in the second or third decade of life, with lesions that are fixed and soft. Biopsy would have confirmed this diagnosis.
Diabetic dermopathy (choice “c”) manifests with atrophic patches on anterior tibial skin. The patches occasionally become superficially eroded but do not resemble this patient’s lesions at all.
Juvenile xanthogranuloma (choice “d”) usually presents on children as a solitary yellowish brown papule. It can resemble eruptive xanthomata histologically but not clinically.
DISCUSSION
Eruptive xanthomata (EX) are relatively common, manifesting rapidly as papules and nodules, most frequently in the setting of hypertriglyceridemia. The latter can be familial and may be worsened by poorly controlled diabetes. Persons with Fredrickson types I, IV, and V hyperlipidemia are especially prone to EX.
As might be expected, patients with EX are at risk for several associated morbidities, including acute pancreatitis (especially in childhood cases) and atherosclerotic vessel disease. EX have also been associated with hypothyroid states and nephrotic syndromes.
Elevations in cholesterol, with normal triglyceride levels, can be associated with several types of xanthoma, including plane xanthomas and xanthelasma. The latter, often benign, can manifest in a normolipemic patient as well (necessitating a problem-directed history, physical, and lipid check).
Biopsy is often required to confirm the diagnosis of EX. As in this case, it typically shows monotonous collections of lipid-laden macrophages. Frozen sections of EX can be successfully stained for lipids, but routine processing of specimens effectively removes any lipids, replacing them with paraffin.
TREATMENT
Treatment entails controlling lipids with medication (fenofibrate), diet, and exercise and getting diabetes under control, as indicated. It is also essential to assess for atherosclerotic vessel disease and rule out pancreatitis.
Within a month of institution of treatment, this patient’s lesions had all but disappeared. His serum amylase and lipase were within normal limits, and testing for atherosclerotic vessel disease was pending.
Click here for more DermaDiagnosis cases, including
• The Value of Certainty in Diagnosis
• A Purplish Rash on the Instep
• Hair Loss at a Very Young Age.
Although they are unaccompanied by any other symptoms, this man is understandably alarmed by the extensive lesions covering much of his body. They first appeared months ago but have become more numerous, larger, and more prominent with time. The patient’s history includes type 2 diabetes (often poorly controlled) and dyslipidemia, for which he takes fenofibrate. Several years ago, he experienced a similar skin outbreak, which resolved after the patient increased his exercise and gained better control of his blood glucose. The condition is striking. There are widespread bilateral collections of shallow intradermal papules, nodules, and plaques primarily on the extensor surfaces of the patient’s arms, legs, and thighs and the convex surfaces of his buttocks. Numbering into the hundreds, the lesions spare his palms, soles, face, and scalp. No abnormality of the periocular skin is appreciated. Moderately firm on palpation, the lesions range in size from 1 to 3 cm in diameter. In several locations, they are linearly configured. A 4-mm punch biopsy of one of them shows large numbers of foamy macrophages in the epidermis and upper dermis. Bloodwork reveals a triglyceride level of 3,850 mg/dL.
Generalized Yellow Discoloration of the Skin
The Diagnosis: Carotenemia
Laboratory parameters including thyroid function testing as well as total protein and bilirubin levels were within reference range. Testing revealed multiple food allergies to almonds, oranges, cashews, garlic, peanuts, and cantaloupe. The patient was treated with a dietary expansion based on his allergy testing.
ß-Carotene converts to vitamin A in the intestine and acts as a lipochrome. Lack of conversion can be noted as an inborn error of metabolism.1 Many green, yellow, and orange fruits and vegetables contain ß-carotene, including carrots, sweet potatoes, squash, green beans, papayas, and pumpkins.1-3 ß-Carotene also is used as a vitamin supplement4 or therapeutic agent in photosensitive disorders such as genetic porphyrias.5
ß-Carotene can accumulate in the stratum corneum and impart a yellow color to the skin when the circulating levels are high; this coloration is termed carotenemia.1,4 Carotenemia is common in infants and young children who have diets rich in green and orange vegetable purees.6 Carotenemia limited to thick areas of the skin, such as the palms and soles, can be seen in adults who eat large amounts of carrots; generalized carotenemia is rare.1,4
Carotenemia is a benign condition of excess cutaneous buildup of ß-carotene through excessive intake of carotene-rich foods1-4 or nutritional supplements7 or through association with anorexia, liver disease, renal disease, hypothyroidism, or diabetes mellitus.1,4,8,9 Carotene deposits usually are most notable in areas with thick stratum corneum, such as the nasolabial folds, palms, and soles, as opposed to areas such as the conjunctivae and mucosa.1,4
Carotenemia may mimic jaundice and should be differentiated through scleral examination for icterus and bilirubin levels. Carotene levels can be tested but generally are unnecessary. Carotenemia can be seen in liver or renal disease and can exacerbate the yellow coloration seen in jaundiced individuals.1,4,9
Because it is a benign condition, the pathology usually is limited to skin discoloration, as seen in our patient. Although this condition can be reversed with a modified diet, our patient had multiple food allergies that further restricted his vegetarian diet, thereby limiting the modifications that he was willing to make to his diet.
1. Schwartz RA. Carotenemia. Emedicine. http://emedicine.medscape.com/article/1104368-overview. Updated April 8, 2014. Accessed April 30, 2014.
2. Sale TA, Stratman E. Carotenemia associated with green bean ingestion. Pediatr Dermatol. 2004;21:657-659.
3. Costanza DJ. Carotenemia associated with papaya ingestion. Calif Med. 1968;109:319-320.
4. Lascari AD. Carotenemia. a review. Clin Pediatr (Phila). 1981;20:25-29.
5. Puy H, Gouya L, Deybach JC. Porphyrias. Lancet. 2010;375:924-937.
6. Karthik SV, Campbell-Davidson D, Isherwood D. Carotenemia in infancy and its association with prevalent feeding practices. Pediatr Dermatol. 2006;23:571-573.
7. Takita Y, Ichimiya M, Hamamoto Y, et al. A case of carotenemia associated with ingestion of nutrient supplements. J Dermatol. 2006;2:132-134.
8. Thibault L, Roberge AG. The nutritional status of subjects with nervosa. Int J Vitam Nutr Res. 1987;57:447-452.
9. Matthews-Roth M, Gulbrandsen CL. Transport of beta-carotene in serum of individuals with carotenemia. Clin Chem. 1974;20:1578-1579.
The Diagnosis: Carotenemia
Laboratory parameters including thyroid function testing as well as total protein and bilirubin levels were within reference range. Testing revealed multiple food allergies to almonds, oranges, cashews, garlic, peanuts, and cantaloupe. The patient was treated with a dietary expansion based on his allergy testing.
ß-Carotene converts to vitamin A in the intestine and acts as a lipochrome. Lack of conversion can be noted as an inborn error of metabolism.1 Many green, yellow, and orange fruits and vegetables contain ß-carotene, including carrots, sweet potatoes, squash, green beans, papayas, and pumpkins.1-3 ß-Carotene also is used as a vitamin supplement4 or therapeutic agent in photosensitive disorders such as genetic porphyrias.5
ß-Carotene can accumulate in the stratum corneum and impart a yellow color to the skin when the circulating levels are high; this coloration is termed carotenemia.1,4 Carotenemia is common in infants and young children who have diets rich in green and orange vegetable purees.6 Carotenemia limited to thick areas of the skin, such as the palms and soles, can be seen in adults who eat large amounts of carrots; generalized carotenemia is rare.1,4
Carotenemia is a benign condition of excess cutaneous buildup of ß-carotene through excessive intake of carotene-rich foods1-4 or nutritional supplements7 or through association with anorexia, liver disease, renal disease, hypothyroidism, or diabetes mellitus.1,4,8,9 Carotene deposits usually are most notable in areas with thick stratum corneum, such as the nasolabial folds, palms, and soles, as opposed to areas such as the conjunctivae and mucosa.1,4
Carotenemia may mimic jaundice and should be differentiated through scleral examination for icterus and bilirubin levels. Carotene levels can be tested but generally are unnecessary. Carotenemia can be seen in liver or renal disease and can exacerbate the yellow coloration seen in jaundiced individuals.1,4,9
Because it is a benign condition, the pathology usually is limited to skin discoloration, as seen in our patient. Although this condition can be reversed with a modified diet, our patient had multiple food allergies that further restricted his vegetarian diet, thereby limiting the modifications that he was willing to make to his diet.
The Diagnosis: Carotenemia
Laboratory parameters including thyroid function testing as well as total protein and bilirubin levels were within reference range. Testing revealed multiple food allergies to almonds, oranges, cashews, garlic, peanuts, and cantaloupe. The patient was treated with a dietary expansion based on his allergy testing.
ß-Carotene converts to vitamin A in the intestine and acts as a lipochrome. Lack of conversion can be noted as an inborn error of metabolism.1 Many green, yellow, and orange fruits and vegetables contain ß-carotene, including carrots, sweet potatoes, squash, green beans, papayas, and pumpkins.1-3 ß-Carotene also is used as a vitamin supplement4 or therapeutic agent in photosensitive disorders such as genetic porphyrias.5
ß-Carotene can accumulate in the stratum corneum and impart a yellow color to the skin when the circulating levels are high; this coloration is termed carotenemia.1,4 Carotenemia is common in infants and young children who have diets rich in green and orange vegetable purees.6 Carotenemia limited to thick areas of the skin, such as the palms and soles, can be seen in adults who eat large amounts of carrots; generalized carotenemia is rare.1,4
Carotenemia is a benign condition of excess cutaneous buildup of ß-carotene through excessive intake of carotene-rich foods1-4 or nutritional supplements7 or through association with anorexia, liver disease, renal disease, hypothyroidism, or diabetes mellitus.1,4,8,9 Carotene deposits usually are most notable in areas with thick stratum corneum, such as the nasolabial folds, palms, and soles, as opposed to areas such as the conjunctivae and mucosa.1,4
Carotenemia may mimic jaundice and should be differentiated through scleral examination for icterus and bilirubin levels. Carotene levels can be tested but generally are unnecessary. Carotenemia can be seen in liver or renal disease and can exacerbate the yellow coloration seen in jaundiced individuals.1,4,9
Because it is a benign condition, the pathology usually is limited to skin discoloration, as seen in our patient. Although this condition can be reversed with a modified diet, our patient had multiple food allergies that further restricted his vegetarian diet, thereby limiting the modifications that he was willing to make to his diet.
1. Schwartz RA. Carotenemia. Emedicine. http://emedicine.medscape.com/article/1104368-overview. Updated April 8, 2014. Accessed April 30, 2014.
2. Sale TA, Stratman E. Carotenemia associated with green bean ingestion. Pediatr Dermatol. 2004;21:657-659.
3. Costanza DJ. Carotenemia associated with papaya ingestion. Calif Med. 1968;109:319-320.
4. Lascari AD. Carotenemia. a review. Clin Pediatr (Phila). 1981;20:25-29.
5. Puy H, Gouya L, Deybach JC. Porphyrias. Lancet. 2010;375:924-937.
6. Karthik SV, Campbell-Davidson D, Isherwood D. Carotenemia in infancy and its association with prevalent feeding practices. Pediatr Dermatol. 2006;23:571-573.
7. Takita Y, Ichimiya M, Hamamoto Y, et al. A case of carotenemia associated with ingestion of nutrient supplements. J Dermatol. 2006;2:132-134.
8. Thibault L, Roberge AG. The nutritional status of subjects with nervosa. Int J Vitam Nutr Res. 1987;57:447-452.
9. Matthews-Roth M, Gulbrandsen CL. Transport of beta-carotene in serum of individuals with carotenemia. Clin Chem. 1974;20:1578-1579.
1. Schwartz RA. Carotenemia. Emedicine. http://emedicine.medscape.com/article/1104368-overview. Updated April 8, 2014. Accessed April 30, 2014.
2. Sale TA, Stratman E. Carotenemia associated with green bean ingestion. Pediatr Dermatol. 2004;21:657-659.
3. Costanza DJ. Carotenemia associated with papaya ingestion. Calif Med. 1968;109:319-320.
4. Lascari AD. Carotenemia. a review. Clin Pediatr (Phila). 1981;20:25-29.
5. Puy H, Gouya L, Deybach JC. Porphyrias. Lancet. 2010;375:924-937.
6. Karthik SV, Campbell-Davidson D, Isherwood D. Carotenemia in infancy and its association with prevalent feeding practices. Pediatr Dermatol. 2006;23:571-573.
7. Takita Y, Ichimiya M, Hamamoto Y, et al. A case of carotenemia associated with ingestion of nutrient supplements. J Dermatol. 2006;2:132-134.
8. Thibault L, Roberge AG. The nutritional status of subjects with nervosa. Int J Vitam Nutr Res. 1987;57:447-452.
9. Matthews-Roth M, Gulbrandsen CL. Transport of beta-carotene in serum of individuals with carotenemia. Clin Chem. 1974;20:1578-1579.
A 50-year-old man presented with yellow, pruritic, xerotic skin and lethargy. The patient also reported nasal congestion and sneezing, especially when eating peanuts. He was fearful of allergic reactions and restricted his diet to “safe foods” such as squash, green beans, and sweet potatoes. On examination the patient had marked generalized yellow discoloration of the skin with pale mucous membranes, nonicteric sclerae, infraocular violaceous and hyperpigmented skin (allergic shiners), and Dennie-Morgan folds.
Pruritus and Hyperpigmented Streaks
The Diagnosis: Flagellate Hyperpigmentation
Physical examination of our patient revealed multiple linear, flagellate, and hyperpigmented plaques on the trunk. Similar lesions were seen on the upper and lower extremities. Given the patient's history of non-Hodgkin lymphoma and concurrent chemotherapy, the use of systemic steroids was avoided. Notable improvement in the symptoms was achieved with topical steroids (triamcinolone acetonide ointment) under occlusion and systemic gabapentin. The patient's chemotherapy is ongoing. This characteristic skin eruption often is seen after treatment with systemic bleomycin sulfate.
Flagellate hyperpigmentation following systemic bleomycin sulfate use as a chemotherapeutic agent is a well-known cutaneous reaction that occurs in approximately 10% of exposed individuals.1 Bleomycin sulfate, an antibiotic derived from Streptomyces verticillus, is a commonly used antitumor agent for both hematologic and solid-organ malignancies.2 Many patients develop generalized pruritus within several days after initiating bleomycin sulfate, followed by the characteristic linear and hyperpigmented streaks. The exact mechanism of this characteristic rash is unknown. Suggested pathways include induction of neutrophilic eccrine hidradenitis, postinflammatory pigmentary incontinence, and altered levels of melanin due to a toxic effect of the medication. Other commonly reported adverse events to bleomycin sulfate include alopecia and stomatitis.3
Although a diffuse rash is the most commonly reported cutaneous reaction to systemic bleomycin sulfate, there have been reports of hyperpigmentation only in areas of pressure and palmar creases. Tsuji and Sawabe4 reported a case of hyperpigmentation limited to areas of striae distensae after systemic bleomycin sulfate. Flagellate hyperpigmentation also has been reported following intralesional bleomycin sulfate for the treatment of verruca plantaris. In that case, the patient had received 14 U of intralesional bleomycin sulfate injected at different sites of recalcitrant verruca and developed urticaria, generalized pruritus, and flagellate hyperpigmentation.5
Treatment of flagellate hyperpigmentation includes cessation of the medication, which is not always possible due to the need for an effective chemotherapeutic regimen. Most cases are reversible following discontinuation of bleomycin sulfate, and care is directed at symptom relief with antipruritic agents, antihistamines, systemic steroids, or topical steroids.3
1. Watanabe T, Tsuchida T. 'Flagellate' erythema in dermatomyositis. Dermatology. 1995;190:230-231.
2. Yagoda A, Mukherji B, Young C, et al. Bleomycin, an antitumor antibiotic. clinical experience in 274 patients. Ann Intern Med. 1972;77:861-870.
3. Susser WS, Whitaker-Worth DL, Grant-Kels JM. Mucocutaneous reactions to chemotherapy. J Am Acad Dermatol. 1999;40:367-398; quiz 399-400.
4. Tsuji T, Sawabe M. Hyperpigmentation in striae distensae after bleomycin treatment. J Am Acad Dermatol. 1993;28:503-505.
5. Abess A, Keel DM, Graham BS. Flagellate hyperpigmentation following intralesional bleomycin treatment of verruca plantaris. Arch Dermatol. 2003;139:337-339.
The Diagnosis: Flagellate Hyperpigmentation
Physical examination of our patient revealed multiple linear, flagellate, and hyperpigmented plaques on the trunk. Similar lesions were seen on the upper and lower extremities. Given the patient's history of non-Hodgkin lymphoma and concurrent chemotherapy, the use of systemic steroids was avoided. Notable improvement in the symptoms was achieved with topical steroids (triamcinolone acetonide ointment) under occlusion and systemic gabapentin. The patient's chemotherapy is ongoing. This characteristic skin eruption often is seen after treatment with systemic bleomycin sulfate.
Flagellate hyperpigmentation following systemic bleomycin sulfate use as a chemotherapeutic agent is a well-known cutaneous reaction that occurs in approximately 10% of exposed individuals.1 Bleomycin sulfate, an antibiotic derived from Streptomyces verticillus, is a commonly used antitumor agent for both hematologic and solid-organ malignancies.2 Many patients develop generalized pruritus within several days after initiating bleomycin sulfate, followed by the characteristic linear and hyperpigmented streaks. The exact mechanism of this characteristic rash is unknown. Suggested pathways include induction of neutrophilic eccrine hidradenitis, postinflammatory pigmentary incontinence, and altered levels of melanin due to a toxic effect of the medication. Other commonly reported adverse events to bleomycin sulfate include alopecia and stomatitis.3
Although a diffuse rash is the most commonly reported cutaneous reaction to systemic bleomycin sulfate, there have been reports of hyperpigmentation only in areas of pressure and palmar creases. Tsuji and Sawabe4 reported a case of hyperpigmentation limited to areas of striae distensae after systemic bleomycin sulfate. Flagellate hyperpigmentation also has been reported following intralesional bleomycin sulfate for the treatment of verruca plantaris. In that case, the patient had received 14 U of intralesional bleomycin sulfate injected at different sites of recalcitrant verruca and developed urticaria, generalized pruritus, and flagellate hyperpigmentation.5
Treatment of flagellate hyperpigmentation includes cessation of the medication, which is not always possible due to the need for an effective chemotherapeutic regimen. Most cases are reversible following discontinuation of bleomycin sulfate, and care is directed at symptom relief with antipruritic agents, antihistamines, systemic steroids, or topical steroids.3
The Diagnosis: Flagellate Hyperpigmentation
Physical examination of our patient revealed multiple linear, flagellate, and hyperpigmented plaques on the trunk. Similar lesions were seen on the upper and lower extremities. Given the patient's history of non-Hodgkin lymphoma and concurrent chemotherapy, the use of systemic steroids was avoided. Notable improvement in the symptoms was achieved with topical steroids (triamcinolone acetonide ointment) under occlusion and systemic gabapentin. The patient's chemotherapy is ongoing. This characteristic skin eruption often is seen after treatment with systemic bleomycin sulfate.
Flagellate hyperpigmentation following systemic bleomycin sulfate use as a chemotherapeutic agent is a well-known cutaneous reaction that occurs in approximately 10% of exposed individuals.1 Bleomycin sulfate, an antibiotic derived from Streptomyces verticillus, is a commonly used antitumor agent for both hematologic and solid-organ malignancies.2 Many patients develop generalized pruritus within several days after initiating bleomycin sulfate, followed by the characteristic linear and hyperpigmented streaks. The exact mechanism of this characteristic rash is unknown. Suggested pathways include induction of neutrophilic eccrine hidradenitis, postinflammatory pigmentary incontinence, and altered levels of melanin due to a toxic effect of the medication. Other commonly reported adverse events to bleomycin sulfate include alopecia and stomatitis.3
Although a diffuse rash is the most commonly reported cutaneous reaction to systemic bleomycin sulfate, there have been reports of hyperpigmentation only in areas of pressure and palmar creases. Tsuji and Sawabe4 reported a case of hyperpigmentation limited to areas of striae distensae after systemic bleomycin sulfate. Flagellate hyperpigmentation also has been reported following intralesional bleomycin sulfate for the treatment of verruca plantaris. In that case, the patient had received 14 U of intralesional bleomycin sulfate injected at different sites of recalcitrant verruca and developed urticaria, generalized pruritus, and flagellate hyperpigmentation.5
Treatment of flagellate hyperpigmentation includes cessation of the medication, which is not always possible due to the need for an effective chemotherapeutic regimen. Most cases are reversible following discontinuation of bleomycin sulfate, and care is directed at symptom relief with antipruritic agents, antihistamines, systemic steroids, or topical steroids.3
1. Watanabe T, Tsuchida T. 'Flagellate' erythema in dermatomyositis. Dermatology. 1995;190:230-231.
2. Yagoda A, Mukherji B, Young C, et al. Bleomycin, an antitumor antibiotic. clinical experience in 274 patients. Ann Intern Med. 1972;77:861-870.
3. Susser WS, Whitaker-Worth DL, Grant-Kels JM. Mucocutaneous reactions to chemotherapy. J Am Acad Dermatol. 1999;40:367-398; quiz 399-400.
4. Tsuji T, Sawabe M. Hyperpigmentation in striae distensae after bleomycin treatment. J Am Acad Dermatol. 1993;28:503-505.
5. Abess A, Keel DM, Graham BS. Flagellate hyperpigmentation following intralesional bleomycin treatment of verruca plantaris. Arch Dermatol. 2003;139:337-339.
1. Watanabe T, Tsuchida T. 'Flagellate' erythema in dermatomyositis. Dermatology. 1995;190:230-231.
2. Yagoda A, Mukherji B, Young C, et al. Bleomycin, an antitumor antibiotic. clinical experience in 274 patients. Ann Intern Med. 1972;77:861-870.
3. Susser WS, Whitaker-Worth DL, Grant-Kels JM. Mucocutaneous reactions to chemotherapy. J Am Acad Dermatol. 1999;40:367-398; quiz 399-400.
4. Tsuji T, Sawabe M. Hyperpigmentation in striae distensae after bleomycin treatment. J Am Acad Dermatol. 1993;28:503-505.
5. Abess A, Keel DM, Graham BS. Flagellate hyperpigmentation following intralesional bleomycin treatment of verruca plantaris. Arch Dermatol. 2003;139:337-339.
A 57-year-old man presented to the dermatology clinic for evaluation of a widespread pruritic rash. The patient had a recent diagnosis of non-Hodgkin lymphoma and was currently undergoing chemotherapy with adriamycin, bleomycin sulfate, vinblastine sulfate, and dacarbazine. The patient’s medical history included hypertension, which was well controlled, and a remote history of a stroke. The patient stated that the rash was recurrent and developed a few days after each dose of bleomycin. Review of systems was otherwise unremarkable, except for pruritus.
