Kristopher M. Peters, DO

The Diagnosis: Cutaneous Amyloidosis

A punch biopsy confirmed the diagnosis of cutaneous amyloidosis, which is characterized by the deposition of amyloid proteins in the skin without systemic involvement. Subtypes of cutaneous amyloidosis include lichenoid, macular, and nodular amyloidosis. A mixed or biphasic amyloidosis can occur when both lichenoid and macular lesions are present.¹ Lichenoid and macular amyloidosis generally are characterized by moderate to severe pruritus. Lichenoid amyloidosis favors the shins, calves, ankles, and extensor extremities; macular amyloidosis has a predilection for the interscapular area and less frequently the upper arms, chest, and thighs.² Atypical variants also have been reported, including amyloidosis cutis dyschromica, poikilodermalike amyloidosis, and bullous amyloidosis, as well as incontinentia pigmenti–like, linear, and nevoid types.³ Macular amyloidosis has been reported to occur in association with progressive systemic sclerosis, primary biliary cirrhosis, systemic lupus erythematosus, paronychia, and multiple endocrine neoplasia type 2.²

Acanthosis nigricans typically presents on the neck and intertriginous areas as velvety hyperpigmented plaques. Confluent and reticulated papillomatosis also appears as slightly elevated papules; however, it occurs in the intermammary region in a reticulated pattern. Ichthyosis vulgaris also may occur on the lower extremities but presents with adherent large scales rather than papules. Keratosis pilaris may present on the proximal lower extremities with smaller, folliculocentric, fleshcolored to pink papules.

Treatment of cutaneous amyloidosis has long been challenging for dermatologists. The primary focus should be treatment of any underlying disease that is causing the pruritus and subsequent manipulation of skin lesions. Topical calcipotriol, phototherapy, oral cyclophosphamide, and Nd:YAG laser have demonstrated beneficial outcomes. IL-31 antibodies may be a potential future treatment.¹

The Diagnosis: Cutaneous Amyloidosis

A punch biopsy confirmed the diagnosis of cutaneous amyloidosis, which is characterized by the deposition of amyloid proteins in the skin without systemic involvement. Subtypes of cutaneous amyloidosis include lichenoid, macular, and nodular amyloidosis. A mixed or biphasic amyloidosis can occur when both lichenoid and macular lesions are present.¹ Lichenoid and macular amyloidosis generally are characterized by moderate to severe pruritus. Lichenoid amyloidosis favors the shins, calves, ankles, and extensor extremities; macular amyloidosis has a predilection for the interscapular area and less frequently the upper arms, chest, and thighs.² Atypical variants also have been reported, including amyloidosis cutis dyschromica, poikilodermalike amyloidosis, and bullous amyloidosis, as well as incontinentia pigmenti–like, linear, and nevoid types.³ Macular amyloidosis has been reported to occur in association with progressive systemic sclerosis, primary biliary cirrhosis, systemic lupus erythematosus, paronychia, and multiple endocrine neoplasia type 2.²

Acanthosis nigricans typically presents on the neck and intertriginous areas as velvety hyperpigmented plaques. Confluent and reticulated papillomatosis also appears as slightly elevated papules; however, it occurs in the intermammary region in a reticulated pattern. Ichthyosis vulgaris also may occur on the lower extremities but presents with adherent large scales rather than papules. Keratosis pilaris may present on the proximal lower extremities with smaller, folliculocentric, fleshcolored to pink papules.

Treatment of cutaneous amyloidosis has long been challenging for dermatologists. The primary focus should be treatment of any underlying disease that is causing the pruritus and subsequent manipulation of skin lesions. Topical calcipotriol, phototherapy, oral cyclophosphamide, and Nd:YAG laser have demonstrated beneficial outcomes. IL-31 antibodies may be a potential future treatment.¹

The Diagnosis: Cutaneous Amyloidosis

A punch biopsy confirmed the diagnosis of cutaneous amyloidosis, which is characterized by the deposition of amyloid proteins in the skin without systemic involvement. Subtypes of cutaneous amyloidosis include lichenoid, macular, and nodular amyloidosis. A mixed or biphasic amyloidosis can occur when both lichenoid and macular lesions are present.¹ Lichenoid and macular amyloidosis generally are characterized by moderate to severe pruritus. Lichenoid amyloidosis favors the shins, calves, ankles, and extensor extremities; macular amyloidosis has a predilection for the interscapular area and less frequently the upper arms, chest, and thighs.² Atypical variants also have been reported, including amyloidosis cutis dyschromica, poikilodermalike amyloidosis, and bullous amyloidosis, as well as incontinentia pigmenti–like, linear, and nevoid types.³ Macular amyloidosis has been reported to occur in association with progressive systemic sclerosis, primary biliary cirrhosis, systemic lupus erythematosus, paronychia, and multiple endocrine neoplasia type 2.²

Acanthosis nigricans typically presents on the neck and intertriginous areas as velvety hyperpigmented plaques. Confluent and reticulated papillomatosis also appears as slightly elevated papules; however, it occurs in the intermammary region in a reticulated pattern. Ichthyosis vulgaris also may occur on the lower extremities but presents with adherent large scales rather than papules. Keratosis pilaris may present on the proximal lower extremities with smaller, folliculocentric, fleshcolored to pink papules.

Treatment of cutaneous amyloidosis has long been challenging for dermatologists. The primary focus should be treatment of any underlying disease that is causing the pruritus and subsequent manipulation of skin lesions. Topical calcipotriol, phototherapy, oral cyclophosphamide, and Nd:YAG laser have demonstrated beneficial outcomes. IL-31 antibodies may be a potential future treatment.¹

The Diagnosis: Favre-Racouchot Syndrome

Favre-Racouchot syndrome, also known as nodular elastosis with cysts and comedones, is seen in approximately 6% of adults aged 40 to 60 years and predominantly is observed in White males.¹ Typically, patients have a history of prolonged recreational or occupational UV exposure and tobacco use. The diagnosis can be made clinically; no biopsy is necessary. If a biopsy is performed, histologic findings typically consist of notable actinic elastosis, epidermal atrophy, and comedones. The differential diagnosis includes acne comedones, colloid milium, milia, chloracne, and trichoepithelioma.² Associated conditions that have been found concurrently include cutis rhomboidalis nuchae, actinic keratosis, erosive pustular dermatosis, actinic granuloma, and basal and squamous cell carcinomas.²

The pathogenesis, while not fully understood, seems to involve a combination of chronic UV radiation exposure and heavy cigarette smoking that eventually leads to cutaneous atrophy and keratinization of the pilosebaceous follicles as well as the formation of comedones.² Radiation therapy also has been implicated as a possible causative agent of Favre-Racouchot syndrome.¹ Clinically, symmetric distribution of large black comedones over the temporal and periorbital areas is seen surrounded by distinct signs of UV-damaged skin, including wrinkles and atrophic skin.³ Although there seems to be a synergistic effect between cigarette smoking and chronic UV exposure, evidence favors smoking as the major cause of this condition,^4,5 which causes striking visual changes but is a benign process. UV protection and smoking cessation are the most important factors for prevention and limiting progression.

Treatment consists of typical comedonal therapies such as tretinoin or comedone extraction. Procedural options in conjunction with medical therapy include dermabrasion or laser therapy. Newer studies have shown promising results for both CO₂ laser treatment and plasma exeresis.⁶ Plasma exeresis is a noninvasive technique that causes ionization of atmospheric gas between the device and tissue, ultimately causing sublimation of the target tissue.⁷ It is important to carefully evaluate and follow up with these patients due to their history of extensive UV exposure. Both short-term and long-term follow-up is recommended due to high rates of reoccurrence within 10 to 12 months and the dangers of chronic UV exposure– related malignancies.⁶

The Diagnosis: Favre-Racouchot Syndrome

Favre-Racouchot syndrome, also known as nodular elastosis with cysts and comedones, is seen in approximately 6% of adults aged 40 to 60 years and predominantly is observed in White males.¹ Typically, patients have a history of prolonged recreational or occupational UV exposure and tobacco use. The diagnosis can be made clinically; no biopsy is necessary. If a biopsy is performed, histologic findings typically consist of notable actinic elastosis, epidermal atrophy, and comedones. The differential diagnosis includes acne comedones, colloid milium, milia, chloracne, and trichoepithelioma.² Associated conditions that have been found concurrently include cutis rhomboidalis nuchae, actinic keratosis, erosive pustular dermatosis, actinic granuloma, and basal and squamous cell carcinomas.²

The pathogenesis, while not fully understood, seems to involve a combination of chronic UV radiation exposure and heavy cigarette smoking that eventually leads to cutaneous atrophy and keratinization of the pilosebaceous follicles as well as the formation of comedones.² Radiation therapy also has been implicated as a possible causative agent of Favre-Racouchot syndrome.¹ Clinically, symmetric distribution of large black comedones over the temporal and periorbital areas is seen surrounded by distinct signs of UV-damaged skin, including wrinkles and atrophic skin.³ Although there seems to be a synergistic effect between cigarette smoking and chronic UV exposure, evidence favors smoking as the major cause of this condition,^4,5 which causes striking visual changes but is a benign process. UV protection and smoking cessation are the most important factors for prevention and limiting progression.

Treatment consists of typical comedonal therapies such as tretinoin or comedone extraction. Procedural options in conjunction with medical therapy include dermabrasion or laser therapy. Newer studies have shown promising results for both CO₂ laser treatment and plasma exeresis.⁶ Plasma exeresis is a noninvasive technique that causes ionization of atmospheric gas between the device and tissue, ultimately causing sublimation of the target tissue.⁷ It is important to carefully evaluate and follow up with these patients due to their history of extensive UV exposure. Both short-term and long-term follow-up is recommended due to high rates of reoccurrence within 10 to 12 months and the dangers of chronic UV exposure– related malignancies.⁶

The Diagnosis: Favre-Racouchot Syndrome

Favre-Racouchot syndrome, also known as nodular elastosis with cysts and comedones, is seen in approximately 6% of adults aged 40 to 60 years and predominantly is observed in White males.¹ Typically, patients have a history of prolonged recreational or occupational UV exposure and tobacco use. The diagnosis can be made clinically; no biopsy is necessary. If a biopsy is performed, histologic findings typically consist of notable actinic elastosis, epidermal atrophy, and comedones. The differential diagnosis includes acne comedones, colloid milium, milia, chloracne, and trichoepithelioma.² Associated conditions that have been found concurrently include cutis rhomboidalis nuchae, actinic keratosis, erosive pustular dermatosis, actinic granuloma, and basal and squamous cell carcinomas.²

The pathogenesis, while not fully understood, seems to involve a combination of chronic UV radiation exposure and heavy cigarette smoking that eventually leads to cutaneous atrophy and keratinization of the pilosebaceous follicles as well as the formation of comedones.² Radiation therapy also has been implicated as a possible causative agent of Favre-Racouchot syndrome.¹ Clinically, symmetric distribution of large black comedones over the temporal and periorbital areas is seen surrounded by distinct signs of UV-damaged skin, including wrinkles and atrophic skin.³ Although there seems to be a synergistic effect between cigarette smoking and chronic UV exposure, evidence favors smoking as the major cause of this condition,^4,5 which causes striking visual changes but is a benign process. UV protection and smoking cessation are the most important factors for prevention and limiting progression.

Treatment consists of typical comedonal therapies such as tretinoin or comedone extraction. Procedural options in conjunction with medical therapy include dermabrasion or laser therapy. Newer studies have shown promising results for both CO₂ laser treatment and plasma exeresis.⁶ Plasma exeresis is a noninvasive technique that causes ionization of atmospheric gas between the device and tissue, ultimately causing sublimation of the target tissue.⁷ It is important to carefully evaluate and follow up with these patients due to their history of extensive UV exposure. Both short-term and long-term follow-up is recommended due to high rates of reoccurrence within 10 to 12 months and the dangers of chronic UV exposure– related malignancies.⁶

To the Editor:

Disseminated cryptococcosis is a well-known opportunistic infection in patients with advanced human immunodeficiency virus (HIV) infection, but it is not frequently seen as a primary infection of the skin in immunocompetent hosts. We report a case of primary cutaneous cryptococcosis (PCC) of the lower legs in an immunocompetent Iraq War veteran.

A 28-year-old female service member presented to the dermatology clinic with progressively enlarging plaquelike lesions on the shins of 6 months’ duration. The patient had resided and worked as a deployed soldier in the lower level of a bullet hole–laden, pigeon-infested observation tower in southern Iraq 9 months prior to the current presentation. During her 7-month deployment, she reported daily exposure to pigeon excreta on equipment and frequently sustained superficial abrasions and lacerations to the legs due to the cramped and hazardous working environment. The patient noticed intensely pruritic, bugbitelike papular lesions on the shins and calves 1 month after residing in the observation tower. She sought medical treatment and was given hydrocortisone cream 1% and calamine lotion for a presumed irritant dermatitis. Over the ensuing 3 months, the pruritus worsened, and the primary lesions coalesced into annular erythematous plaques (Figure).

After returning to the United States, the patient presented again for medical care and was given ketoconazole cream 1% for presumed tinea corporis, which resulted in no improvement. A dermatologic consultation and evaluation ensued with subsequent microbial workup showing no bacterial growth on wound culture and no fungal elements on a potassium hydroxide preparation. Hematoxylin and eosin, periodic acid–Schiff, and Grocott-Gomori methenamine-silver staining did not demonstrate any organisms. Tissue cultures for bacteria and acid-fast bacilli showed no growth. A fungal tissue culture ultimately confirmed the presence of Cryptococcus neoformans. A lumbar puncture showed no evidence of Cryptococcus on DNA probe testing. Serologic testing for HIV was negative, and brain magnetic resonance imaging showed no lesions. Sputum culture and staining showed no fungal elements, and a chest radiograph was normal. A diagnosis of PCC was made and therapy with oral fluconazole 200 mg twice daily was initiated, with the intention of completing a 6-month course. During the treatment, the pruritus resolved within 3 weeks and the lesions involuted over 3 months. From the time of onset of the lesions throughout treatment, the patient showed no pulmonary, neurologic, or other systemic symptoms. She currently is healthy with no evidence of recurrence.

Primary cutaneous cryptococcosis mainly affects individuals with underlying immunosuppression, most commonly due to advanced HIV, prolonged treatment with immunosuppressive medications, or organ transplantation.¹ The most common route of inoculation is by inhalation of Cryptococcus spores with subsequent hematogenous dissemination.² Primary cutaneous cryptococcosis with skin lesions and no concomitant systemic involvement has rarely been reported, and when encountered, it usually is associated with environments that predispose patients to skin wounds with simultaneous exposure to soil or vegetative debris contaminated with bird excreta.³ Primary cutaneous cryptococcosis can present in a myriad of ways, including papules, nodules, plaques, and even necrotizing fasciitis–like skin lesions.^4,5 This variable presentation often creates clinical confusion and diagnostic delay; therefore, a high index of suspicion is required for timely diagnosis.

Due to the worldwide deployment of US military service members, exotic cutaneous infectious diseases such as PCC may be encountered in dermatology practice. Prompt clinical and histologic diagnosis is imperative to assess for systemic disease and avoid cutaneous spread and morbidity in US service members and travelers returning home from the Middle East.

To the Editor:

Disseminated cryptococcosis is a well-known opportunistic infection in patients with advanced human immunodeficiency virus (HIV) infection, but it is not frequently seen as a primary infection of the skin in immunocompetent hosts. We report a case of primary cutaneous cryptococcosis (PCC) of the lower legs in an immunocompetent Iraq War veteran.

A 28-year-old female service member presented to the dermatology clinic with progressively enlarging plaquelike lesions on the shins of 6 months’ duration. The patient had resided and worked as a deployed soldier in the lower level of a bullet hole–laden, pigeon-infested observation tower in southern Iraq 9 months prior to the current presentation. During her 7-month deployment, she reported daily exposure to pigeon excreta on equipment and frequently sustained superficial abrasions and lacerations to the legs due to the cramped and hazardous working environment. The patient noticed intensely pruritic, bugbitelike papular lesions on the shins and calves 1 month after residing in the observation tower. She sought medical treatment and was given hydrocortisone cream 1% and calamine lotion for a presumed irritant dermatitis. Over the ensuing 3 months, the pruritus worsened, and the primary lesions coalesced into annular erythematous plaques (Figure).

After returning to the United States, the patient presented again for medical care and was given ketoconazole cream 1% for presumed tinea corporis, which resulted in no improvement. A dermatologic consultation and evaluation ensued with subsequent microbial workup showing no bacterial growth on wound culture and no fungal elements on a potassium hydroxide preparation. Hematoxylin and eosin, periodic acid–Schiff, and Grocott-Gomori methenamine-silver staining did not demonstrate any organisms. Tissue cultures for bacteria and acid-fast bacilli showed no growth. A fungal tissue culture ultimately confirmed the presence of Cryptococcus neoformans. A lumbar puncture showed no evidence of Cryptococcus on DNA probe testing. Serologic testing for HIV was negative, and brain magnetic resonance imaging showed no lesions. Sputum culture and staining showed no fungal elements, and a chest radiograph was normal. A diagnosis of PCC was made and therapy with oral fluconazole 200 mg twice daily was initiated, with the intention of completing a 6-month course. During the treatment, the pruritus resolved within 3 weeks and the lesions involuted over 3 months. From the time of onset of the lesions throughout treatment, the patient showed no pulmonary, neurologic, or other systemic symptoms. She currently is healthy with no evidence of recurrence.

Primary cutaneous cryptococcosis mainly affects individuals with underlying immunosuppression, most commonly due to advanced HIV, prolonged treatment with immunosuppressive medications, or organ transplantation.¹ The most common route of inoculation is by inhalation of Cryptococcus spores with subsequent hematogenous dissemination.² Primary cutaneous cryptococcosis with skin lesions and no concomitant systemic involvement has rarely been reported, and when encountered, it usually is associated with environments that predispose patients to skin wounds with simultaneous exposure to soil or vegetative debris contaminated with bird excreta.³ Primary cutaneous cryptococcosis can present in a myriad of ways, including papules, nodules, plaques, and even necrotizing fasciitis–like skin lesions.^4,5 This variable presentation often creates clinical confusion and diagnostic delay; therefore, a high index of suspicion is required for timely diagnosis.

Due to the worldwide deployment of US military service members, exotic cutaneous infectious diseases such as PCC may be encountered in dermatology practice. Prompt clinical and histologic diagnosis is imperative to assess for systemic disease and avoid cutaneous spread and morbidity in US service members and travelers returning home from the Middle East.

To the Editor:

Disseminated cryptococcosis is a well-known opportunistic infection in patients with advanced human immunodeficiency virus (HIV) infection, but it is not frequently seen as a primary infection of the skin in immunocompetent hosts. We report a case of primary cutaneous cryptococcosis (PCC) of the lower legs in an immunocompetent Iraq War veteran.

A 28-year-old female service member presented to the dermatology clinic with progressively enlarging plaquelike lesions on the shins of 6 months’ duration. The patient had resided and worked as a deployed soldier in the lower level of a bullet hole–laden, pigeon-infested observation tower in southern Iraq 9 months prior to the current presentation. During her 7-month deployment, she reported daily exposure to pigeon excreta on equipment and frequently sustained superficial abrasions and lacerations to the legs due to the cramped and hazardous working environment. The patient noticed intensely pruritic, bugbitelike papular lesions on the shins and calves 1 month after residing in the observation tower. She sought medical treatment and was given hydrocortisone cream 1% and calamine lotion for a presumed irritant dermatitis. Over the ensuing 3 months, the pruritus worsened, and the primary lesions coalesced into annular erythematous plaques (Figure).

After returning to the United States, the patient presented again for medical care and was given ketoconazole cream 1% for presumed tinea corporis, which resulted in no improvement. A dermatologic consultation and evaluation ensued with subsequent microbial workup showing no bacterial growth on wound culture and no fungal elements on a potassium hydroxide preparation. Hematoxylin and eosin, periodic acid–Schiff, and Grocott-Gomori methenamine-silver staining did not demonstrate any organisms. Tissue cultures for bacteria and acid-fast bacilli showed no growth. A fungal tissue culture ultimately confirmed the presence of Cryptococcus neoformans. A lumbar puncture showed no evidence of Cryptococcus on DNA probe testing. Serologic testing for HIV was negative, and brain magnetic resonance imaging showed no lesions. Sputum culture and staining showed no fungal elements, and a chest radiograph was normal. A diagnosis of PCC was made and therapy with oral fluconazole 200 mg twice daily was initiated, with the intention of completing a 6-month course. During the treatment, the pruritus resolved within 3 weeks and the lesions involuted over 3 months. From the time of onset of the lesions throughout treatment, the patient showed no pulmonary, neurologic, or other systemic symptoms. She currently is healthy with no evidence of recurrence.

Primary cutaneous cryptococcosis mainly affects individuals with underlying immunosuppression, most commonly due to advanced HIV, prolonged treatment with immunosuppressive medications, or organ transplantation.¹ The most common route of inoculation is by inhalation of Cryptococcus spores with subsequent hematogenous dissemination.² Primary cutaneous cryptococcosis with skin lesions and no concomitant systemic involvement has rarely been reported, and when encountered, it usually is associated with environments that predispose patients to skin wounds with simultaneous exposure to soil or vegetative debris contaminated with bird excreta.³ Primary cutaneous cryptococcosis can present in a myriad of ways, including papules, nodules, plaques, and even necrotizing fasciitis–like skin lesions.^4,5 This variable presentation often creates clinical confusion and diagnostic delay; therefore, a high index of suspicion is required for timely diagnosis.

Due to the worldwide deployment of US military service members, exotic cutaneous infectious diseases such as PCC may be encountered in dermatology practice. Prompt clinical and histologic diagnosis is imperative to assess for systemic disease and avoid cutaneous spread and morbidity in US service members and travelers returning home from the Middle East.