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Indian Pediatr 2012;49: 80

Angiokeratoma Circumscriptum Neviforme


Panchami Debbarman, Sudipta Roy and Piyush Kumar

Department of Dermatology, Katihar Medical College, Katihar, Bihar.
Email: [email protected]

 


A 10 years-old boy presented with multiple small bluish-red papules on the scrotum for the past 2 years. These lesions were gradually increasing in number and had a tendency to bleed after minor trauma. On examination, multiple discrete smooth surfaced bluish-red papules were found unilaterally on scrotum. (Fig. 1) Palpation ruled out varicocele or inguinal hernia. Rest of the mucocutaneous examination was unremarkable. Skin biopsy from a papule showed mild hyperkeratosis with large, numerous, dilated capillaries in papillary dermis. USG pelvis and scrotum were done to rule out any vascular anomaly. Considering clinical feature and histopathology, the patient was diagnosed as angiokeratoma circumscriptum neviforme (ACN).

Fig. 1 Blue-red grouped papules present unilaterally on scrotum (A). Close up of lesions (B).

Angiokeratomas are characterized by asymptomatic, 2-5 mm, blue red hyperkeratotic papules. Histologically, they are composed of ectatic thin-walled vessels in the superficial dermis with overlying epidermal hyperplasia. Angiokeratoma can be localized or generalized. The generalized form, angiokeratoma corporis diffusum, is usually associated with a metabolic disorder, the most common being Fabry disease. The localized forms are solitary angiokeratoma (typically occurs on the legs and follows trauma), localized angiokeratoma of the scrotum and vulva (Fordyce type), Mibelli type (bilateral angiokeratomas on the dorsa of the fingers and toes), and angiokeratoma circumscriptum neviforme (multiple, hyperkeratotic, papular and plaque like lesions, usually unilaterally on the lower leg, foot, and buttock). The major differential diagnosis is angiokeratoma Fordyce type (middle aged persons, bilateral lesions) and lymphan-gioma circumscriptum (yellowish grouped vesicles containing clear fluid). Herpes zoster and herpes simplex (short duration) too need to be differentiated. The principal problems are intermittent bleeding, anxiety, and over-treatment due to misdiagnosis. It is treated by various locally destructive modalities including electrocoagu-lation, excision, cryotherapy, or LASER therapy.

 

 

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