Irregularly Hyperpigmented Plaque on the Right Heel

Article Type
Changed
Thu, 02/21/2019 - 11:24
Display Headline
Irregularly Hyperpigmented Plaque on the Right Heel

The Diagnosis: Pigmented Bowen Disease

A biopsy of the lesion was performed for suspected acral malignant melanoma. Hematoxylin and eosin staining revealed acanthosis, elongation of rete ridges, and keratinocytes in complete disorder with atypical mitoses and pleomorphism affecting the full layer of the epidermis (Figure 1). The basement membrane was intact. Melanin pigmentation was increased in the lower epidermis and the upper dermis, and a lymphohistiocytic inflammatory infiltrate was present in the dermis. Staining for carcinoembryonic antigen (Figure 2) and melanoma
antigen (Figure 3) recognized by T cells (melan-A) both revealed negative results. Histopathologic findings led to the diagnosis of pigmented Bowen disease (BD).

Figure 1. Keratinocytes in complete disorder with atypical mitoses and pleomorphism affecting the full layer of the epidermis, along with increased melanin pigmentation in the lower epidermis and the upper dermis (H&E, original magnification ×400).

Figure 2. Staining for carcinoembryonic antigen was negative (original magnification ×200).

Figure 3. Staining for melanoma antigen recognized by T cells was negative (original magnification ×200).

Pigmented BD is a rare variant that accounts for 1.7% (N=420) to 5.5% (N=951) of all cases of BD.1,2 It is reported to affect men more than women and to be more prevalent in individuals with higher Fitzpatrick skin types.3 Furthermore, exposure to UV radiation, chemicals (eg, arsenic), or human papillomavirus, as well as immunosuppression, are known to be related to pigmented BD.2,4 Clinically, pigmented BD commonly involves nonexposed areas such as the anogenital area, trunk, and extremities, unlike typical BD that involves sun-exposed areas.5 In addition, it most frequently presents as a well-delineated, irregularly pigmented, asymptomatic
plaque and not as a scaly erythematous plaque. Therefore, the clinical diagnosis may be challenging. The differential diagnosis includes malignant melanoma, pigmented extramammary Paget disease, pigmented basal cell carcinoma, seborrheic keratosis, pigmented actinic keratosis, solar lentigo, and melanocytic nevi.

Histopathologically, a varying amount of melanin deposit is noted on hematoxylin and eosin staining, along with features of BD, including disarrayed atypical keratinocytes involving the full epidermis but not the basement membrane, with atypical individual cell keratinization.3,5,6 Pigmented extramammary Paget disease can mimic pigmented BD clinically and pathologically, but Paget cells stain positive for anticytokeratin (CAM 5.2), carcinoembryonic antigen, and mucicarmine, whereas cells in pigmented BD stain negative.7 Moreover, negative staining for human melanoma black, melan-A, and S-100 helps differentiate malignant melanoma from pigmented BD.8

The prognosis of pigmented BD is similar to classic BD and is independent of the presence of melanin pigment.6 Therefore, the treatment options do not differ from those for typical BD and include surgical excision, cryotherapy, laser ablation, topical imiquimod or 5-fluorouracil, curettage, electrosurgery, and photodynamic therapy (PDT).

In our case, the patient and her family did not want surgical removal; therefore, 1 course of fractional laser-assisted PDT and 2 courses of ablative laser-assisted PDT were performed. Unfortunately, the lesion persisted, possibly because it was too large and pigmented. Two months later, ingenol mebutate gel 0.05% was applied (4 courses) after using an ablative laser over 3 consecutive days with a 1-month interval between courses. The lesion resolved without any adverse events.

References
  1. Cameron A, Rosendahl C, Tschandl P, et al. Dermatoscopy of pigmented Bowen’s disease [published online January 15, 2010]. J Am Acad Dermatol. 2010;62:597-604.
  2. Ragi G, Turner MS, Klein LE, et al. Pigmented Bowen’s disease and review of 420 Bowen’s disease lesions. J Dermatol Surg Oncol. 1988;14:765-769.
  3. Hernandez C, Ivkovic A, Fowler A. Growing plaque on foot. J Fam Pract. 2008;57:603-605.
  4. Hwang SW, Kim JW, Park SW, et al. Two cases of pigmented Bowen’s disease. Ann Dermatol 2002;14:127-129.
  5. Wilmer EM, Lee KC, Higgins W 2nd, et al. Hyperpigmented palmar plaque: an unexpected diagnosis of Bowen disease. Dermatol Online J. 2013;19:18573.
  6. Brinca A, Teixeira V, Gonçalo M, et al. A large pigmented lesion mimicking malignant melanoma. Clin Exp Dermatol. 2012;37:817-884.
  7. Hilliard NJ, Huang C, Andea A. Pigmented extramammary Paget’s disease of the axilla mimicking melanoma: case report and review of the literature. J Cutan Pathol. 2009;36:995-1000.
  8. Öztürk Durmaz E, Dog˘ an Ekici I, Ozian F, et al. Pigmented Bowen’s disease of the genitalia masquerading as malignant melanoma. Acta Dermatovenerol Croat. 2015;23:130-133.
Article PDF
Author and Disclosure Information

From the Department of Dermatology, Ajou University School of Medicine, Suwon, Korea.

The authors report no conflict of interest.

Correspondence: You Chan Kim, MD, PhD, Department of Dermatology, Ajou University School of Medicine, 164 Worldcup-ro, Yeongtong-gu, Suwon, Korea, 16499 (maychan@ajou.ac.kr).

Issue
Cutis - 103(2)
Publications
Topics
Page Number
E13-E15
Sections
Author and Disclosure Information

From the Department of Dermatology, Ajou University School of Medicine, Suwon, Korea.

The authors report no conflict of interest.

Correspondence: You Chan Kim, MD, PhD, Department of Dermatology, Ajou University School of Medicine, 164 Worldcup-ro, Yeongtong-gu, Suwon, Korea, 16499 (maychan@ajou.ac.kr).

Author and Disclosure Information

From the Department of Dermatology, Ajou University School of Medicine, Suwon, Korea.

The authors report no conflict of interest.

Correspondence: You Chan Kim, MD, PhD, Department of Dermatology, Ajou University School of Medicine, 164 Worldcup-ro, Yeongtong-gu, Suwon, Korea, 16499 (maychan@ajou.ac.kr).

Article PDF
Article PDF
Related Articles

The Diagnosis: Pigmented Bowen Disease

A biopsy of the lesion was performed for suspected acral malignant melanoma. Hematoxylin and eosin staining revealed acanthosis, elongation of rete ridges, and keratinocytes in complete disorder with atypical mitoses and pleomorphism affecting the full layer of the epidermis (Figure 1). The basement membrane was intact. Melanin pigmentation was increased in the lower epidermis and the upper dermis, and a lymphohistiocytic inflammatory infiltrate was present in the dermis. Staining for carcinoembryonic antigen (Figure 2) and melanoma
antigen (Figure 3) recognized by T cells (melan-A) both revealed negative results. Histopathologic findings led to the diagnosis of pigmented Bowen disease (BD).

Figure 1. Keratinocytes in complete disorder with atypical mitoses and pleomorphism affecting the full layer of the epidermis, along with increased melanin pigmentation in the lower epidermis and the upper dermis (H&E, original magnification ×400).

Figure 2. Staining for carcinoembryonic antigen was negative (original magnification ×200).

Figure 3. Staining for melanoma antigen recognized by T cells was negative (original magnification ×200).

Pigmented BD is a rare variant that accounts for 1.7% (N=420) to 5.5% (N=951) of all cases of BD.1,2 It is reported to affect men more than women and to be more prevalent in individuals with higher Fitzpatrick skin types.3 Furthermore, exposure to UV radiation, chemicals (eg, arsenic), or human papillomavirus, as well as immunosuppression, are known to be related to pigmented BD.2,4 Clinically, pigmented BD commonly involves nonexposed areas such as the anogenital area, trunk, and extremities, unlike typical BD that involves sun-exposed areas.5 In addition, it most frequently presents as a well-delineated, irregularly pigmented, asymptomatic
plaque and not as a scaly erythematous plaque. Therefore, the clinical diagnosis may be challenging. The differential diagnosis includes malignant melanoma, pigmented extramammary Paget disease, pigmented basal cell carcinoma, seborrheic keratosis, pigmented actinic keratosis, solar lentigo, and melanocytic nevi.

Histopathologically, a varying amount of melanin deposit is noted on hematoxylin and eosin staining, along with features of BD, including disarrayed atypical keratinocytes involving the full epidermis but not the basement membrane, with atypical individual cell keratinization.3,5,6 Pigmented extramammary Paget disease can mimic pigmented BD clinically and pathologically, but Paget cells stain positive for anticytokeratin (CAM 5.2), carcinoembryonic antigen, and mucicarmine, whereas cells in pigmented BD stain negative.7 Moreover, negative staining for human melanoma black, melan-A, and S-100 helps differentiate malignant melanoma from pigmented BD.8

The prognosis of pigmented BD is similar to classic BD and is independent of the presence of melanin pigment.6 Therefore, the treatment options do not differ from those for typical BD and include surgical excision, cryotherapy, laser ablation, topical imiquimod or 5-fluorouracil, curettage, electrosurgery, and photodynamic therapy (PDT).

In our case, the patient and her family did not want surgical removal; therefore, 1 course of fractional laser-assisted PDT and 2 courses of ablative laser-assisted PDT were performed. Unfortunately, the lesion persisted, possibly because it was too large and pigmented. Two months later, ingenol mebutate gel 0.05% was applied (4 courses) after using an ablative laser over 3 consecutive days with a 1-month interval between courses. The lesion resolved without any adverse events.

The Diagnosis: Pigmented Bowen Disease

A biopsy of the lesion was performed for suspected acral malignant melanoma. Hematoxylin and eosin staining revealed acanthosis, elongation of rete ridges, and keratinocytes in complete disorder with atypical mitoses and pleomorphism affecting the full layer of the epidermis (Figure 1). The basement membrane was intact. Melanin pigmentation was increased in the lower epidermis and the upper dermis, and a lymphohistiocytic inflammatory infiltrate was present in the dermis. Staining for carcinoembryonic antigen (Figure 2) and melanoma
antigen (Figure 3) recognized by T cells (melan-A) both revealed negative results. Histopathologic findings led to the diagnosis of pigmented Bowen disease (BD).

Figure 1. Keratinocytes in complete disorder with atypical mitoses and pleomorphism affecting the full layer of the epidermis, along with increased melanin pigmentation in the lower epidermis and the upper dermis (H&E, original magnification ×400).

Figure 2. Staining for carcinoembryonic antigen was negative (original magnification ×200).

Figure 3. Staining for melanoma antigen recognized by T cells was negative (original magnification ×200).

Pigmented BD is a rare variant that accounts for 1.7% (N=420) to 5.5% (N=951) of all cases of BD.1,2 It is reported to affect men more than women and to be more prevalent in individuals with higher Fitzpatrick skin types.3 Furthermore, exposure to UV radiation, chemicals (eg, arsenic), or human papillomavirus, as well as immunosuppression, are known to be related to pigmented BD.2,4 Clinically, pigmented BD commonly involves nonexposed areas such as the anogenital area, trunk, and extremities, unlike typical BD that involves sun-exposed areas.5 In addition, it most frequently presents as a well-delineated, irregularly pigmented, asymptomatic
plaque and not as a scaly erythematous plaque. Therefore, the clinical diagnosis may be challenging. The differential diagnosis includes malignant melanoma, pigmented extramammary Paget disease, pigmented basal cell carcinoma, seborrheic keratosis, pigmented actinic keratosis, solar lentigo, and melanocytic nevi.

Histopathologically, a varying amount of melanin deposit is noted on hematoxylin and eosin staining, along with features of BD, including disarrayed atypical keratinocytes involving the full epidermis but not the basement membrane, with atypical individual cell keratinization.3,5,6 Pigmented extramammary Paget disease can mimic pigmented BD clinically and pathologically, but Paget cells stain positive for anticytokeratin (CAM 5.2), carcinoembryonic antigen, and mucicarmine, whereas cells in pigmented BD stain negative.7 Moreover, negative staining for human melanoma black, melan-A, and S-100 helps differentiate malignant melanoma from pigmented BD.8

The prognosis of pigmented BD is similar to classic BD and is independent of the presence of melanin pigment.6 Therefore, the treatment options do not differ from those for typical BD and include surgical excision, cryotherapy, laser ablation, topical imiquimod or 5-fluorouracil, curettage, electrosurgery, and photodynamic therapy (PDT).

In our case, the patient and her family did not want surgical removal; therefore, 1 course of fractional laser-assisted PDT and 2 courses of ablative laser-assisted PDT were performed. Unfortunately, the lesion persisted, possibly because it was too large and pigmented. Two months later, ingenol mebutate gel 0.05% was applied (4 courses) after using an ablative laser over 3 consecutive days with a 1-month interval between courses. The lesion resolved without any adverse events.

References
  1. Cameron A, Rosendahl C, Tschandl P, et al. Dermatoscopy of pigmented Bowen’s disease [published online January 15, 2010]. J Am Acad Dermatol. 2010;62:597-604.
  2. Ragi G, Turner MS, Klein LE, et al. Pigmented Bowen’s disease and review of 420 Bowen’s disease lesions. J Dermatol Surg Oncol. 1988;14:765-769.
  3. Hernandez C, Ivkovic A, Fowler A. Growing plaque on foot. J Fam Pract. 2008;57:603-605.
  4. Hwang SW, Kim JW, Park SW, et al. Two cases of pigmented Bowen’s disease. Ann Dermatol 2002;14:127-129.
  5. Wilmer EM, Lee KC, Higgins W 2nd, et al. Hyperpigmented palmar plaque: an unexpected diagnosis of Bowen disease. Dermatol Online J. 2013;19:18573.
  6. Brinca A, Teixeira V, Gonçalo M, et al. A large pigmented lesion mimicking malignant melanoma. Clin Exp Dermatol. 2012;37:817-884.
  7. Hilliard NJ, Huang C, Andea A. Pigmented extramammary Paget’s disease of the axilla mimicking melanoma: case report and review of the literature. J Cutan Pathol. 2009;36:995-1000.
  8. Öztürk Durmaz E, Dog˘ an Ekici I, Ozian F, et al. Pigmented Bowen’s disease of the genitalia masquerading as malignant melanoma. Acta Dermatovenerol Croat. 2015;23:130-133.
References
  1. Cameron A, Rosendahl C, Tschandl P, et al. Dermatoscopy of pigmented Bowen’s disease [published online January 15, 2010]. J Am Acad Dermatol. 2010;62:597-604.
  2. Ragi G, Turner MS, Klein LE, et al. Pigmented Bowen’s disease and review of 420 Bowen’s disease lesions. J Dermatol Surg Oncol. 1988;14:765-769.
  3. Hernandez C, Ivkovic A, Fowler A. Growing plaque on foot. J Fam Pract. 2008;57:603-605.
  4. Hwang SW, Kim JW, Park SW, et al. Two cases of pigmented Bowen’s disease. Ann Dermatol 2002;14:127-129.
  5. Wilmer EM, Lee KC, Higgins W 2nd, et al. Hyperpigmented palmar plaque: an unexpected diagnosis of Bowen disease. Dermatol Online J. 2013;19:18573.
  6. Brinca A, Teixeira V, Gonçalo M, et al. A large pigmented lesion mimicking malignant melanoma. Clin Exp Dermatol. 2012;37:817-884.
  7. Hilliard NJ, Huang C, Andea A. Pigmented extramammary Paget’s disease of the axilla mimicking melanoma: case report and review of the literature. J Cutan Pathol. 2009;36:995-1000.
  8. Öztürk Durmaz E, Dog˘ an Ekici I, Ozian F, et al. Pigmented Bowen’s disease of the genitalia masquerading as malignant melanoma. Acta Dermatovenerol Croat. 2015;23:130-133.
Issue
Cutis - 103(2)
Issue
Cutis - 103(2)
Page Number
E13-E15
Page Number
E13-E15
Publications
Publications
Topics
Article Type
Display Headline
Irregularly Hyperpigmented Plaque on the Right Heel
Display Headline
Irregularly Hyperpigmented Plaque on the Right Heel
Sections
Questionnaire Body

 

A 56-year-old woman presented with an asymptomatic plaque on the right heel that had grown
steadily over the last year. Pigmented lesions were not appreciated on other sites, and lymph nodes were not enlarged. Her medical history was otherwise normal, except for bilateral hearing loss due to encephalitis at the age of 5 years. None of her family members had similar symptoms. Physical examination revealed a well-defined, irregularly hyperpigmented plaque on the right heel.

Disallow All Ads
Content Gating
No Gating (article Unlocked/Free)
Alternative CME
Disqus Comments
Default
Use ProPublica
CFC Schedule Remove Status
Tue, 01/08/2019 - 11:00
Article PDF Media

What Is Your Diagnosis? Acquired Lymphangiectasia

Article Type
Changed
Thu, 01/10/2019 - 13:21
Display Headline
What Is Your Diagnosis? Acquired Lymphangiectasia

A 19-year-old woman presented with an umbilical mass of 5 months’ duration that had grown in size. Physical examination revealed a 1×1-cm brownish, pedunculated, cauliflower-shaped lesion on the umbilicus. There were no other signs or symptoms of disease. The patient’s personal and family disease history were unremarkable. An excisional biopsy was performed.

The Diagnosis: Acquired Lymphangiectasia

On histopathology numerous dilated channels lined by a single flat layer of endothelial cells were noted within the dermis. The overlying epidermis was papillomatous and acanthotic (Figure 1). The endothelial cells lining the dilated channels were D2-40 positive (Figure 2). Furthermore, the channels contained a pinkish amorphous material and a few red blood cells. The surrounding stroma showed scattered lymphocyte infiltration. These findings were consistent with lymphangiectasia. The lesion has not recurred 4 years following total excision.

Figure 1. Numerous dilated channels lined by flattened, single-layer endothelial cells in the dermis with overlying epidermal papillomatosis and acanthosis (A and B)(H&E, original magnification ×40 and ×200).

Figure 2. The flattened, single-layer endothelial cells that lined multiple dilated channels were positive on D2-40 immunochemical staining (original magnifi-cation ×200).

Acquired lymphangiectasia is known by various names, including lymphangioma, acquired lymphangioma, and acquired lymphangioma circumscriptum, which has led to confusion.1 Acquired lymphangiectasia, which is characterized by dilated superficial lymphatics, develops following damage to previously normal lymphatic channels, leading to a buildup of lymph pressure and backflow.2 Acquired lymphangiectasia has been reported as clinically and histologically indistinguishable from lymphangioma circumscriptum2; however, unlike in lymphangiectasia, the suffix -oma denotes a tumor. Our case matched more closely with the typical concept of lymphangiectasia rather than lymphangioma.

Clinical findings of acquired lymphangiectasia usually include translucent, flat or slightly raised, 2- to 5-mm, flesh-colored papules and vesicles.3,4 Acquired lymphangiectasia has been described with lesions that have verrucous surfaces mimicking warts, condyloma acuminata, or molluscum contagiosum.5,6 Our case suggests that acquired lymphangiectasia also can present with a pedunculated cauliflowerlike appearance. In general, it develops secondary to certain conditions such as recovery from trauma or surgery, postsurgical fibrosis, and irradiation. Lymphangiectasia often is seen on the arms, axillae, chest wall, and genital area in women and the scrotum, penis, thighs, and pubic region in men, both who have undergone radical surgery and irradiation for treatment of breast and prostate cancer, respectively.3 Our patient did not report any history of trauma to the umbilicus.

On histopathology acquired lymphangiectasia typically shows edematous polypoid nodules with dilated lymphatics. The overlying epidermis usually shows a spectrum of proliferation ranging from mild acanthosis to florid pseudoepitheliomatous hyperplasia with marked hyperkeratosis and parakeratosis. The distinctive finding of lymphangiectasia is the presence of dilated lymphatic spaces within the dermis. The dilated channels are filled with lymphatic fluid and often red and white blood cells. The single layer of flattened endothelial cells generally exhibits immunoreactivity to D2-40 and CD31.1

Treatment of lymphangiectasia is focused on reducing the pressure within the lymph vessels and managing consequent lymphedema with compression dressings. Simple surgical excision of lesions on sites such as the vulva or legs often is effective.3 If surgical intervention is not an option, cryotherapy, sclerotherapy, cauterization, and treatment with CO2 lasers also have been utilized with good outcomes.7 In the current case, total surgical excision was performed, which provided good results.

References

1. Stewart CJ, Chan T, Platten M. Acquired lymphangiectasia (‘lymphangioma circumscriptum’) of the vulva: a report of eight cases. Pathology. 2009;41:448-453.

2. Celis AV, Gaughf CN, Sangueza OP, et al. Acquired lymphangiectasis. South Med J. 1999;92:69-72.

3. Verma SB. Lymphangiectasias of the skin: victims of confusing nomenclature. Clin Exp Dermatol. 2009;34:566-569.

4. Mortimer PS. Disorder of lymphatic vessels. In: Burns T, Breathnach S, Cox N, et al, eds. Rook’s Textbook of Dermatology. Vol 3. 8th ed. Hoboken, NJ: Wiley-Blackwell; 2010:48.28-48.29.

5. Sharma R, Tomar S, Chandra M. Acquired vulval lymphangiectases mimicking genital warts. Indian J Dermatol Venereol Leprol. 2002;68:166-167.

6. Horn LC, Kühndel K, Pawlowitsch T, et al. Acquired lymphangioma circumscriptum of the vulva mimicking genital warts. Eur J Obstet Gynecol Reprod Biol. 2005;123:118-120.

7. Patel GA, Schwartz RA. Cutaneous lymphangioma circumscriptum: frog spawn on the skin. Int J Dermatol. 2009;48:1290-1295.

Article PDF
Author and Disclosure Information

Dong Jun Lee, MD; Soo-Eun Jung, MD; You Chan Kim, MD, PhD

From the Department of Dermatology, Ajou University School of Medicine, Suwon, South Korea.

The authors report no conflict of interest.

Correspondence: You Chan Kim, MD, PhD, Department of Dermatology, Ajou University School of Medicine, 5 Wonchon-Dong, Yeongtong-Gu, Suwon 443-721, South Korea (maychan@ajou.ac.kr).

Issue
Cutis - 95(2)
Publications
Topics
Page Number
67, 89-90
Legacy Keywords
lymphangioma, acquired lymphangioma, acquired lymphangioma circumscriptum, lymphatic channels, umbilical lesion
Sections
Author and Disclosure Information

Dong Jun Lee, MD; Soo-Eun Jung, MD; You Chan Kim, MD, PhD

From the Department of Dermatology, Ajou University School of Medicine, Suwon, South Korea.

The authors report no conflict of interest.

Correspondence: You Chan Kim, MD, PhD, Department of Dermatology, Ajou University School of Medicine, 5 Wonchon-Dong, Yeongtong-Gu, Suwon 443-721, South Korea (maychan@ajou.ac.kr).

Author and Disclosure Information

Dong Jun Lee, MD; Soo-Eun Jung, MD; You Chan Kim, MD, PhD

From the Department of Dermatology, Ajou University School of Medicine, Suwon, South Korea.

The authors report no conflict of interest.

Correspondence: You Chan Kim, MD, PhD, Department of Dermatology, Ajou University School of Medicine, 5 Wonchon-Dong, Yeongtong-Gu, Suwon 443-721, South Korea (maychan@ajou.ac.kr).

Article PDF
Article PDF
Related Articles

A 19-year-old woman presented with an umbilical mass of 5 months’ duration that had grown in size. Physical examination revealed a 1×1-cm brownish, pedunculated, cauliflower-shaped lesion on the umbilicus. There were no other signs or symptoms of disease. The patient’s personal and family disease history were unremarkable. An excisional biopsy was performed.

The Diagnosis: Acquired Lymphangiectasia

On histopathology numerous dilated channels lined by a single flat layer of endothelial cells were noted within the dermis. The overlying epidermis was papillomatous and acanthotic (Figure 1). The endothelial cells lining the dilated channels were D2-40 positive (Figure 2). Furthermore, the channels contained a pinkish amorphous material and a few red blood cells. The surrounding stroma showed scattered lymphocyte infiltration. These findings were consistent with lymphangiectasia. The lesion has not recurred 4 years following total excision.

Figure 1. Numerous dilated channels lined by flattened, single-layer endothelial cells in the dermis with overlying epidermal papillomatosis and acanthosis (A and B)(H&E, original magnification ×40 and ×200).

Figure 2. The flattened, single-layer endothelial cells that lined multiple dilated channels were positive on D2-40 immunochemical staining (original magnifi-cation ×200).

Acquired lymphangiectasia is known by various names, including lymphangioma, acquired lymphangioma, and acquired lymphangioma circumscriptum, which has led to confusion.1 Acquired lymphangiectasia, which is characterized by dilated superficial lymphatics, develops following damage to previously normal lymphatic channels, leading to a buildup of lymph pressure and backflow.2 Acquired lymphangiectasia has been reported as clinically and histologically indistinguishable from lymphangioma circumscriptum2; however, unlike in lymphangiectasia, the suffix -oma denotes a tumor. Our case matched more closely with the typical concept of lymphangiectasia rather than lymphangioma.

Clinical findings of acquired lymphangiectasia usually include translucent, flat or slightly raised, 2- to 5-mm, flesh-colored papules and vesicles.3,4 Acquired lymphangiectasia has been described with lesions that have verrucous surfaces mimicking warts, condyloma acuminata, or molluscum contagiosum.5,6 Our case suggests that acquired lymphangiectasia also can present with a pedunculated cauliflowerlike appearance. In general, it develops secondary to certain conditions such as recovery from trauma or surgery, postsurgical fibrosis, and irradiation. Lymphangiectasia often is seen on the arms, axillae, chest wall, and genital area in women and the scrotum, penis, thighs, and pubic region in men, both who have undergone radical surgery and irradiation for treatment of breast and prostate cancer, respectively.3 Our patient did not report any history of trauma to the umbilicus.

On histopathology acquired lymphangiectasia typically shows edematous polypoid nodules with dilated lymphatics. The overlying epidermis usually shows a spectrum of proliferation ranging from mild acanthosis to florid pseudoepitheliomatous hyperplasia with marked hyperkeratosis and parakeratosis. The distinctive finding of lymphangiectasia is the presence of dilated lymphatic spaces within the dermis. The dilated channels are filled with lymphatic fluid and often red and white blood cells. The single layer of flattened endothelial cells generally exhibits immunoreactivity to D2-40 and CD31.1

Treatment of lymphangiectasia is focused on reducing the pressure within the lymph vessels and managing consequent lymphedema with compression dressings. Simple surgical excision of lesions on sites such as the vulva or legs often is effective.3 If surgical intervention is not an option, cryotherapy, sclerotherapy, cauterization, and treatment with CO2 lasers also have been utilized with good outcomes.7 In the current case, total surgical excision was performed, which provided good results.

A 19-year-old woman presented with an umbilical mass of 5 months’ duration that had grown in size. Physical examination revealed a 1×1-cm brownish, pedunculated, cauliflower-shaped lesion on the umbilicus. There were no other signs or symptoms of disease. The patient’s personal and family disease history were unremarkable. An excisional biopsy was performed.

The Diagnosis: Acquired Lymphangiectasia

On histopathology numerous dilated channels lined by a single flat layer of endothelial cells were noted within the dermis. The overlying epidermis was papillomatous and acanthotic (Figure 1). The endothelial cells lining the dilated channels were D2-40 positive (Figure 2). Furthermore, the channels contained a pinkish amorphous material and a few red blood cells. The surrounding stroma showed scattered lymphocyte infiltration. These findings were consistent with lymphangiectasia. The lesion has not recurred 4 years following total excision.

Figure 1. Numerous dilated channels lined by flattened, single-layer endothelial cells in the dermis with overlying epidermal papillomatosis and acanthosis (A and B)(H&E, original magnification ×40 and ×200).

Figure 2. The flattened, single-layer endothelial cells that lined multiple dilated channels were positive on D2-40 immunochemical staining (original magnifi-cation ×200).

Acquired lymphangiectasia is known by various names, including lymphangioma, acquired lymphangioma, and acquired lymphangioma circumscriptum, which has led to confusion.1 Acquired lymphangiectasia, which is characterized by dilated superficial lymphatics, develops following damage to previously normal lymphatic channels, leading to a buildup of lymph pressure and backflow.2 Acquired lymphangiectasia has been reported as clinically and histologically indistinguishable from lymphangioma circumscriptum2; however, unlike in lymphangiectasia, the suffix -oma denotes a tumor. Our case matched more closely with the typical concept of lymphangiectasia rather than lymphangioma.

Clinical findings of acquired lymphangiectasia usually include translucent, flat or slightly raised, 2- to 5-mm, flesh-colored papules and vesicles.3,4 Acquired lymphangiectasia has been described with lesions that have verrucous surfaces mimicking warts, condyloma acuminata, or molluscum contagiosum.5,6 Our case suggests that acquired lymphangiectasia also can present with a pedunculated cauliflowerlike appearance. In general, it develops secondary to certain conditions such as recovery from trauma or surgery, postsurgical fibrosis, and irradiation. Lymphangiectasia often is seen on the arms, axillae, chest wall, and genital area in women and the scrotum, penis, thighs, and pubic region in men, both who have undergone radical surgery and irradiation for treatment of breast and prostate cancer, respectively.3 Our patient did not report any history of trauma to the umbilicus.

On histopathology acquired lymphangiectasia typically shows edematous polypoid nodules with dilated lymphatics. The overlying epidermis usually shows a spectrum of proliferation ranging from mild acanthosis to florid pseudoepitheliomatous hyperplasia with marked hyperkeratosis and parakeratosis. The distinctive finding of lymphangiectasia is the presence of dilated lymphatic spaces within the dermis. The dilated channels are filled with lymphatic fluid and often red and white blood cells. The single layer of flattened endothelial cells generally exhibits immunoreactivity to D2-40 and CD31.1

Treatment of lymphangiectasia is focused on reducing the pressure within the lymph vessels and managing consequent lymphedema with compression dressings. Simple surgical excision of lesions on sites such as the vulva or legs often is effective.3 If surgical intervention is not an option, cryotherapy, sclerotherapy, cauterization, and treatment with CO2 lasers also have been utilized with good outcomes.7 In the current case, total surgical excision was performed, which provided good results.

References

1. Stewart CJ, Chan T, Platten M. Acquired lymphangiectasia (‘lymphangioma circumscriptum’) of the vulva: a report of eight cases. Pathology. 2009;41:448-453.

2. Celis AV, Gaughf CN, Sangueza OP, et al. Acquired lymphangiectasis. South Med J. 1999;92:69-72.

3. Verma SB. Lymphangiectasias of the skin: victims of confusing nomenclature. Clin Exp Dermatol. 2009;34:566-569.

4. Mortimer PS. Disorder of lymphatic vessels. In: Burns T, Breathnach S, Cox N, et al, eds. Rook’s Textbook of Dermatology. Vol 3. 8th ed. Hoboken, NJ: Wiley-Blackwell; 2010:48.28-48.29.

5. Sharma R, Tomar S, Chandra M. Acquired vulval lymphangiectases mimicking genital warts. Indian J Dermatol Venereol Leprol. 2002;68:166-167.

6. Horn LC, Kühndel K, Pawlowitsch T, et al. Acquired lymphangioma circumscriptum of the vulva mimicking genital warts. Eur J Obstet Gynecol Reprod Biol. 2005;123:118-120.

7. Patel GA, Schwartz RA. Cutaneous lymphangioma circumscriptum: frog spawn on the skin. Int J Dermatol. 2009;48:1290-1295.

References

1. Stewart CJ, Chan T, Platten M. Acquired lymphangiectasia (‘lymphangioma circumscriptum’) of the vulva: a report of eight cases. Pathology. 2009;41:448-453.

2. Celis AV, Gaughf CN, Sangueza OP, et al. Acquired lymphangiectasis. South Med J. 1999;92:69-72.

3. Verma SB. Lymphangiectasias of the skin: victims of confusing nomenclature. Clin Exp Dermatol. 2009;34:566-569.

4. Mortimer PS. Disorder of lymphatic vessels. In: Burns T, Breathnach S, Cox N, et al, eds. Rook’s Textbook of Dermatology. Vol 3. 8th ed. Hoboken, NJ: Wiley-Blackwell; 2010:48.28-48.29.

5. Sharma R, Tomar S, Chandra M. Acquired vulval lymphangiectases mimicking genital warts. Indian J Dermatol Venereol Leprol. 2002;68:166-167.

6. Horn LC, Kühndel K, Pawlowitsch T, et al. Acquired lymphangioma circumscriptum of the vulva mimicking genital warts. Eur J Obstet Gynecol Reprod Biol. 2005;123:118-120.

7. Patel GA, Schwartz RA. Cutaneous lymphangioma circumscriptum: frog spawn on the skin. Int J Dermatol. 2009;48:1290-1295.

Issue
Cutis - 95(2)
Issue
Cutis - 95(2)
Page Number
67, 89-90
Page Number
67, 89-90
Publications
Publications
Topics
Article Type
Display Headline
What Is Your Diagnosis? Acquired Lymphangiectasia
Display Headline
What Is Your Diagnosis? Acquired Lymphangiectasia
Legacy Keywords
lymphangioma, acquired lymphangioma, acquired lymphangioma circumscriptum, lymphatic channels, umbilical lesion
Legacy Keywords
lymphangioma, acquired lymphangioma, acquired lymphangioma circumscriptum, lymphatic channels, umbilical lesion
Sections
Article Source

PURLs Copyright

Inside the Article

Article PDF Media

Atypical Presentation of Secondary Syphilis

Article Type
Changed
Thu, 01/10/2019 - 13:11
Display Headline
Atypical Presentation of Secondary Syphilis
Article PDF
Issue
Cutis - 92(5)
Publications
Page Number
E5-E6
Legacy Keywords
syphilis, erythema multiform, annular erythematous patch, sexually transmitted diseases and dermatology, erythema, stds and the skin
Sections
Article PDF
Article PDF
Related Articles
Issue
Cutis - 92(5)
Issue
Cutis - 92(5)
Page Number
E5-E6
Page Number
E5-E6
Publications
Publications
Article Type
Display Headline
Atypical Presentation of Secondary Syphilis
Display Headline
Atypical Presentation of Secondary Syphilis
Legacy Keywords
syphilis, erythema multiform, annular erythematous patch, sexually transmitted diseases and dermatology, erythema, stds and the skin
Legacy Keywords
syphilis, erythema multiform, annular erythematous patch, sexually transmitted diseases and dermatology, erythema, stds and the skin
Sections
Disallow All Ads
Alternative CME
Article PDF Media