Congenital atrophic dermatofibrosarcoma protuberans

A 16‐year‐old girl presented with a congenital atrophic plaque on the anterior aspect of her right leg. The lesion had slowly enlarged until she was 9 years of age; subsequently, several tumors appeared on the surface and later became reddish, hot, and mildly painful. Within a few months, all the tumors flattened and involuted. A previous biopsy showed a congenital fibroma. The patient was in good health; the physical examination, chest X‐ray films, and laboratory studies were normal. No history of personal or familial diseases was recorded. Initially, the atrophic plaque measured 6 × 8 cm; shortly after, multiple 0.5–2 cm tumors appeared. These tumors alternated with atrophic and ill‐defined livedo‐like areas ( Fig. 1). A satellite lesion, measuring 2 cm in diameter, was also observed. A new biopsy revealed dermatofibrosarcoma protuberans. 1(A ) 6 × 8 cm plaque on the anterior aspect of the right leg. Multiple tumors alternate with atrophic and ill‐defined livedo‐like areas Immunohistochemical findings included positive vimentin and CD34, and negative Factor XIIIa, S‐100 protein, and muscle actin ( Fig. 2). One month later, several tumors had almost disappeared. The patient was successfully treated with Mohs' micrographic surgery and remained disease free 9 months later. 2The cells are characteristically arranged in a ‘‘storiform’' pattern, sometimes centered around small blood vessels. Involvement of the subcutaneous fat is usual