The diffuse and macular atrophic dermatosis. A precocious and progressive light degenerative condition.
Two brothers with the "diffuse and macular atrophic dermatosis" making a total of 4 patients are reported. The familial occurrence, numerous large "peau citréine" plaques situated on the dorsum of the upper trunk and congenital phimosis with superficial atrophy of glans penis, represent and additional features reported. The light and electron microscopic findings of the least affected site on the arm, of a "peau citréine" and of an erythemoscleroatrophic plaque, none previously studied, have been reported. Among other items, it was also found that the elastic stain-positive globe-like structures found in an unusually large number throughout the edematous upper dermis represent more advancely transformed "Civatte bodies" containing epidermal filamentous cell material.
D000328:Adult; D001284:Atrophy / Q000235:genetics; D006801:Humans; D008297:Male; D010688:Phimosis / Q000235:genetics; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000235:genetics*; D013472:Sunlight