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March 1965

Angiokeratoma Corporis Diffusum (Fabry's Disease)

Author Affiliations


Fellow in Dermatology, Mayo Foundation (Dr. von Gemmingen); Section of Dermatology, Mayo Clinic (Dr. Kierland); Assistant Professor of Pediatrics and Medical Genetics, University of Wisconsin (Dr. Opitz).

Arch Dermatol. 1965;91(3):206-218. doi:10.1001/archderm.1965.01600090014003

A pedigree which includes five males and one female with clinical evidence of angiokeratoma corporis diffusum universale (ACD) is presented. The pattern of involvement lends support to a sex-linked, recessive mode of transmission. ACD cutaneous lesions were examined by histochemical methods, and the results and conclusions are discussed. Cutaneous vasomotor and sudomotor reactions to intradermally injected chemical agents are recorded. The finding of a peculiar histiocytic cell in the bone marrow aspirate is described.

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