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Conjugal Porphyria Cutanea Tarda

Conjugal Porphyria Cutanea Tarda Abstract Porphyria cutanea tarda (PCT), the most common porphyria, may be sporadic or inherited as an autosomal dominant trait and is characterized by decreased activity of the enzyme uroporphyrinogen decarboxylase. Porphyria cutanea tarda may be induced by environmental factors (alcohol, estrogens, iron, and polyhalogenated aromatic hydrocarbons). Associations of PCT with hepatitis B,1,2 hepatitis C,3,4 and human immunodeficiency virus have been reported. We describe an unusual coincidence of PCT in a married couple. Report of Cases. Our patients are a nonconsanguineous husband and wife with similar clinical presentations and porphyrin profiles.A 36-year-old white woman and a 45-year-old white man presented with histories of skin fragility, increasing facial hair, darkening of urine, and blistering of light exposed skin. Neither had a history of hepatitis or of receiving blood transfusions; however, both had used intravenous drugs for 10 years prior to 1980, and had also intermittently ingested moderate-to-heavy amounts of alcohol References 1. Rocchi E, Gilbertini P, Cassanell M, Pietrangeli A, Jensen J. Hepatitis B in porphyria cutanea tarda . Liver . 1986;6:153-157. 2. Valls V, Enriquez de Salamanca R, Lapena L, et al. Hepatitis B serum markers in porphyria cutanea tarda . J Dermatol. 1986;12:24-29. 3. Lacour JPH, Bodokh I, Castanet J, Bekri S, Ortonne JP. Porphyria cutanea tarda and antibodies to hepatitis C virus . Br J Dermatol. 1993;128:121-123.Crossref 4. Herrero C, Vicente A, Bruguera M, et al. Is hepatitis C virus infection a trigger of porphyria cutanea tarda? Lancet. 1993;41:788-789.Crossref 5. Kushner JP, Steinmuller WP, Lee GR. The role of iron in the pathogenesis of PCT: inhibition of uroporphyrinogen decarboxylase . J Clin Invest. 1975;56: 661-667.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1995 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1995.01690130115030
Publisher site
See Article on Publisher Site

Abstract

Abstract Porphyria cutanea tarda (PCT), the most common porphyria, may be sporadic or inherited as an autosomal dominant trait and is characterized by decreased activity of the enzyme uroporphyrinogen decarboxylase. Porphyria cutanea tarda may be induced by environmental factors (alcohol, estrogens, iron, and polyhalogenated aromatic hydrocarbons). Associations of PCT with hepatitis B,1,2 hepatitis C,3,4 and human immunodeficiency virus have been reported. We describe an unusual coincidence of PCT in a married couple. Report of Cases. Our patients are a nonconsanguineous husband and wife with similar clinical presentations and porphyrin profiles.A 36-year-old white woman and a 45-year-old white man presented with histories of skin fragility, increasing facial hair, darkening of urine, and blistering of light exposed skin. Neither had a history of hepatitis or of receiving blood transfusions; however, both had used intravenous drugs for 10 years prior to 1980, and had also intermittently ingested moderate-to-heavy amounts of alcohol References 1. Rocchi E, Gilbertini P, Cassanell M, Pietrangeli A, Jensen J. Hepatitis B in porphyria cutanea tarda . Liver . 1986;6:153-157. 2. Valls V, Enriquez de Salamanca R, Lapena L, et al. Hepatitis B serum markers in porphyria cutanea tarda . J Dermatol. 1986;12:24-29. 3. Lacour JPH, Bodokh I, Castanet J, Bekri S, Ortonne JP. Porphyria cutanea tarda and antibodies to hepatitis C virus . Br J Dermatol. 1993;128:121-123.Crossref 4. Herrero C, Vicente A, Bruguera M, et al. Is hepatitis C virus infection a trigger of porphyria cutanea tarda? Lancet. 1993;41:788-789.Crossref 5. Kushner JP, Steinmuller WP, Lee GR. The role of iron in the pathogenesis of PCT: inhibition of uroporphyrinogen decarboxylase . J Clin Invest. 1975;56: 661-667.Crossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Jan 1, 1995

References