A Rare Case of Acroangiodermatitis Associated with a Congenital Arteriovenous Malformation (Stewart-Bluefarb Syndrome) in a Young Veteran: Case Report and Review of the Literature.

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.

Acrokeratosis Verruciformis of Hopf: a localized variant.

Acrokeratosis Verruciformis of Hopf (AKV) is a rare autosomal dominant genodermatosis. It usually develops during early childhood affecting both sexes equally. Typically, the lesions are warty to convex, brownish to skin-colored papules on the dorsa of the hands and feet, forearms and legs. A possible relationship with Darier disease has long been postulated on a clinical basis mainly. But recently, evidence has suggested that AKV and Darier disease are allelic disorders. I am reporting these two unrelated cases as a unilateral localized variant of AKV.

Acroqueratosis verruciforme diagnosticada en un adulto / Acrokeratosis verruciformis diagnosed in an adult

Se presenta un paciente de 45 años de edad, oficial de las Fuerzas Armadas Revolucionarias (FAR) que acude a Consulta Externa de Dermatología por presentar un cuadro dermatológico de 10 años de evolución sin ningún síntoma subjetivo al inicio de la enfermedad. Basado en su cuadro clínico se planteó la posibilidad de una enfermedad genética. Se confeccionó árbol genealógico y se le realizó biopsia de pie. Se corroboró el diagnóstico de una acroqueratosis verruciforme que fue tratada con criocirugía. Se hace énfasis en la importancia de la profundización del diagnóstico en los exámenes de control y salud a oficiales afectados de dermatosis(AU)

Acroangiodermatitis (pseudo-Kaposi's sarcoma) associated with Klippel-Trénaunay syndrome.

Acroangiodermatitis is an unusual cutaneous sequela of chronic venous insufficiency and congenital vascular malformations of the lower extremities. It typically manifests as purple-colored plaques on the extensor surfaces of the toes of the affected limb. Histologically, it closely resembles Kaposi's sarcoma and had thus been named "pseudo-Kaposi's sarcoma." This is a report of acroangiodermatitis in a patient with Klippel-Trénaunay syndrome.