Acroangiodermatitis (AD) is a rare angioproliferative disease manifesting with cutaneous lesions clinically similar to
Kaposi's sarcoma. AD is a benign
hyperplasia of preexisting vasculature and may be associated with acquired or congenital
arteriovenous malformations (AVM), or severe chronic
venous insufficiency (because of hypostasis, elevated venous pressure, arteriovenous shunting). Stewart-Bluefarb syndrome is the rare syndrome in which AD is associated with a congenital AVM. We present the case of a young veteran with a painful, chronic nonhealing
ulcer and ipsilateral popliteal artery occlusion likely because of
trauma, who elected transmetatarsal
amputation for symptomatic relief. A 24-year-old male veteran presented with a 5-year history of a nonhealing dorsal left
foot ulcer, resulting from a training exercise injury. He ultimately developed
osteomyelitis requiring
antibiotics, frequent
debridements, multiple trials of unsuccessful
skin substitute grafting, and severe unremitting
pain. He noted a remote history of left digital
deformities treated surgically as a child, and an AVM, previously endovascularly treated at an outside facility. Arterial duplex revealed somewhat dampened left popliteal, posterior tibial (PT), and dorsalis pedis (DP) artery signals with arterial brachial index of 1.0. CT angiography showed occlusion of the proximal to mid popliteal artery with significant calcifications felt initially to be a result of prior
trauma. Pedal pulses were palpable and transcutaneous
oxygen measurements revealed adequate oxygenation. Because of unremitting
pain, the patient opted for
amputation. Pathology revealed vascular proliferation consistent with AD. This case illustrates an unusual diagnosis of acroangiodermatitis, and a rare syndrome when associated with his underlying AVM (Stewart-Bluefarb syndrome). This resulted in a painful, chronic
ulcer and was further complicated by
trauma-related
arterial occlusive disease. AD disease can hinder wound healing even in the presence of clinically evident blood flow. Although rare, such unusual diagnoses should be entertained particularly in the unusually young vascular surgical patient.