Misdiagnosis of Fabry disease: importance of biochemical confirmation of clinical or pathological suspicion.

Linthorst, G E; De Rie, M A; Tjiam, K H; Aerts, J M F G; Dingemans, K P; Hollak, C E M · Br J Dermatol · 2004

case_report · Level V

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Abstract

Generalized angiokeratoma are associated with three lysosomal storage disorders, one of which is Fabry disease (alpha-galactosidase A deficiency). Treatment for Fabry disease with supplementation of recombinant enzyme is available in the European Union and subsequently physicians' awareness may rise. A patient who was erroneously diagnosed with Fabry disease is presented.

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