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July 1978

Society Transactions

Arch Dermatol. 1978;114(7):1101. doi:10.1001/archderm.1978.01640190079046

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Waine C. Johnson, MD, Recorder 

Angiokeratoma Corporis Diffusum (Fabry's Disease). Presented by Raul Fleischmajer, MD, and Steven Binnick, MD  This 48-year-old man has a five-year history of nontender, nonpruritic, red papules on his suprapubic area and scrotum. He has had recurrent febrile episodes since infancy accompanied by polyarthralgias and chills lasting about four hours. He had a myocardial infarction in 1974 and again in 1975. Proteinuria developed two years ago and has rapidly increased. He has noted that sweating is limited to the palms and soles. Family history reveals the patient had two siblings who died of "Bright's disease" at ages 32 and 42 years. His first cousin received a kidney transplant after acute renal failure. One son died at the age of 18 months with cystic fibrosis and another son, now 20 years old, has cystic fibrosis.Physical examination disclosed pinhead-sized slightly keratotic reddish papules on the scrotum and pinpoint-sized petechiae on the crural

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