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Article
December 1966

Erythropoietic Protoporphyria (Antea Lipoid Proteinosis) in SistersA Therapeutic Trial With Adenosine Monophosphate

Author Affiliations

MADISON, WIS

From the Institute of Dermatology, St. John's Hospital for Diseases of the Skin, London, and the Department of Dermatology, University of Wisconsin Medical Center, Madison.

Arch Dermatol. 1966;94(6):682-686. doi:10.1001/archderm.1966.01600300006002
Abstract

Two sisters with erythropoietic protoporphyria were given adenosine monophosphate (AMP; adenosine-5-monophosphate; My-B-Den) as a therapeutic trial for five months. This medication failed to produce a significant decrease in the high level of erythrocyte, plasma, or stool protoporphyrins or prevent the recurrence of photosensitivity. In both patients, a considerable natural variation of these porphyrin determinations was observed. The variation in the quantity of plasma and erythrocyte protoporphyrin appeared to be directly related but independent of the fecal protoporphyrin. These cases were previously reported as examples of lipoid proteinosis, principally because of the histological presence of hyalinosis cutis; although this was limited to the light-exposed areas of skin. There have been 150 cases of lipoid proteinosis (hyalinosis cutis et mucosae) described, of which six have now been identified as erythropoietic protoporphyria.

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