Abstract: The porphyrias result from genetic and environmentally induced defects in the heme biosynthetic pathway that leads to the abnormal accumulation of photosensitizing porphyrins. Porphyrias associated with vesiculobullous skin lesion are discussed in this article; this group of disorders includes congenital erythropoietic porphyria, porphyria cutanea tarda, hepatoerythropoietic porphyria, hereditary coproporphyria, and variegate porphyria. Following a review of the heme biosynthetic pathway and consideration of the possible pathophysiology of the cutaneous lesions in the vesiculobullous porphyrias, these entities are addressed individually with regard to cause, clinical manifestations, laboratory findings, differential diagnosis, and treatment.
Publication Year: 1993
Publication Date: 1993-07-01
Language: en
Type: article
Indexed In: ['crossref']
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Cited By Count: 13
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