Angiokeratoma circumscriptum in a young male

Abstract

A 20-year-old male presented with multiple eruptions on his right leg since birth; these bled and were painful on trivial trauma. Examination revealed dark brown, hyperkeratotic, indurated, verrucous linear plaques with irregular borders. Histopathological evidence of hyperkeratosis, acanthosis, and extensive vascular proliferation in papillary dermis confirmed clinical suspicion of angiokeratoma circumscriptum (AKC). Excision and skin grafting yielded a cosmetically favorable outcome. Angiokeratomas, first described by Mibeli in 1889, are a group of vascular ectasias involving the papillary dermis. Angiokeratomas are more common in males; however, AKC-the rarest of its five variants-exhibits a female preponderance (F:M:3:1). AKC is an extremely rare nevoid disorder, only 100 of its cases having been reported in the world literature until 2006. Herein, we have reported a typical case of AKC in a young male that was previously misdiagnosed, and the patient wrongly counseled about the likelihood of its spontaneous regression.

Keywords: Angiokeratoma circumscriptum; vascular malformation; verrucous hemangioma.

Figure 1

(a) Photomicrograph (H and E stain, ×10) of skin biopsy from the right leg demonstrating hyperkeratosis, acanthosis and congestion in the papillary dermis. (b) Photomicrograph (H and E stain, ×40) of skin biopsy from the right leg demonstrating plump endothelial cells in papillary dermis

Figure 2

Linear, multiple, dark brown, hyperkeratotic plaques with irregular borders on the right shin which bleed on trivial trauma

Figure 3

Lesions after excision and skin grafting