Familial atrophie blanche-like lesions with subcutaneous fibrinoid vasculitis. The Georgian ulcers.
Atrophie blanche is an uncommon condition characterized by the development of white atrophic patches of skin on the lower extremities, which form as a result of fibrinoid vasculitis of superficial and mid-dermal vessels followed by necrosis and ulceration of the epidermis. We report four cases in which similar lesions developed on the legs and ankles of young Jewish Russian immigrants to Israel. Although the lesions share many features with atrophie blanche, they differ in their early age of onset, the male predilection, and the extension of the fibrinoid vasculitic process into the subcutaneous tissue. Additionally, the peculiar population clustering (Georgia, U.S.S.R.), common ethnic background, and a family history of similar lesions in close relatives seem to point to a familial or genetic predisposition underlying the development of the disease.
D000293:Adolescent; D000328:Adult; D001284:Atrophy; D001706:Biopsy; D002196:Capillaries / Q000473:pathology; D005260:Female; D005337:Fibrin / Q000378:metabolism; D006801:Humans; D007871:Leg Ulcer / Q000235:genetics / Q000473:pathology; D008297:Male; D009336:Necrosis; D012867:Skin / Q000473:pathology*; D012883:Skin Ulcer / Q000235:genetics* / Q000473:pathology; D014657:Vasculitis / Q000235:genetics* / Q000473:pathology
D005337:Fibrin