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September 1982

Severe Cutaneous Porphyria in a 12-Year-Old BoyHepatoerythropoietic or Symptomatic Porphyria?

Author Affiliations

From the University of Cape Town-Medical Research Council Porphyria Research Unit, Departments of Medicine (Dr Day) and Dermatology (Dr Strauss), University of Cape Town and Groote Schuur Hospital, Cape Town, South Africa.

Arch Dermatol. 1982;118(9):663-667. doi:10.1001/archderm.1982.01650210043016

• Deforming cutaneous porphyria developed in a 12-year-old boy; the severity of his skin disease resolved spontaneously as he grew older. The clinical and biochemical aspects of this case clearly fit the descriptions in the medical literature of both hepatoerythropoietic porphyria (HEP) and infantile symptomatic porphyria (SP). Further porphyrin analyses indicated that our patient suffered from infantile SP. The lack of unquestionably unique features diagnostic for HEP raises strong doubts that it exists as a separate disease entity substantially different from infantile SP.

(Arch Dermatol 1982;118:663-667)