Kaposiform acro-angiodermatitis with arteriovenous malformation (Stewart-Bluefarb syndrome)

A case of acro-angiodermatitis with congenital arteriovenous fistulae (Stewart-Bluefarb syndrome) at the distal right foot in a young man is reported. The lesion evolved slowly from a small dark-red spot existing from birth. Besides mechanical disturbances, the patient's only symptoms were a warmer and somewhat bigger right foot compared to the left nonaffected side. He presented a circumscribed spongy, livid-red coloured angiomatous lesion at the upper part of the distal right foot, also involving the proximal parts of the three middle toes. The diagnosis was based on the clinical aspect and the findings by Doppler ultrasonographic and oscillographic examination. The transcutaneous oxygen pressure showed a marked hypoxia at the edge of the acro-angiodermatitis. Finally, arteriovenous fistulae were demonstrated by arteriography.