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Hereditary uroporphyrinogen-decarboxylase deficiency predisposing porphyria cutanea tarda (chronic hepatic porphyria) in females after oral contraceptive medication.
Arch Dermatol Res. 1985; 278(1):13-6.AD

Abstract

Porphyria cutanea tarda (PCT) was diagnosed in 27 women aged 23-48 years (mean, 35 years) who had been under oral-hormonal-contraceptive medication for 1-18 years, in 3 women under substitutional estrogen treatment in the menopause, and in 2 men aged 65 and 76 years after estrogen treatment of prostatic carcinoma. In all patients, total urinary porphyrin excretion was elevated, with an average uro- and heptacarboxyporphyrin predominance of 88%, thus proving PCT. Of the patients, 84% showed a significant decrease of erythrocyte uroporphyrinogen-decarboxylase (UD; EC 4.1.1.37) activity to approximately 50% of control levels suggesting a hereditary predisposition for the development of a chronic hepatic porphyria. Estrogens and alcohol are capable of reducing hepatic UD activity. Women with hereditary red cell UD deficiency may be regarded as predisposed to PCT when under estrogen intake, especially in combination with the potentiating influence of alcohol and chronic liver disease. Normal erythrocyte UD values in patients with additive alcohol consumption may implicate a stronger inhibitory effect for alcohol on UD, suggesting a merely toxic form of chronic hepatic porphyria.

Authors

No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

4096525

Citation

Sixel-Dietrich, F, and M Doss. "Hereditary Uroporphyrinogen-decarboxylase Deficiency Predisposing Porphyria Cutanea Tarda (chronic Hepatic Porphyria) in Females After Oral Contraceptive Medication." Archives of Dermatological Research, vol. 278, no. 1, 1985, pp. 13-6.
Sixel-Dietrich F, Doss M. Hereditary uroporphyrinogen-decarboxylase deficiency predisposing porphyria cutanea tarda (chronic hepatic porphyria) in females after oral contraceptive medication. Arch Dermatol Res. 1985;278(1):13-6.
Sixel-Dietrich, F., & Doss, M. (1985). Hereditary uroporphyrinogen-decarboxylase deficiency predisposing porphyria cutanea tarda (chronic hepatic porphyria) in females after oral contraceptive medication. Archives of Dermatological Research, 278(1), 13-6.
Sixel-Dietrich F, Doss M. Hereditary Uroporphyrinogen-decarboxylase Deficiency Predisposing Porphyria Cutanea Tarda (chronic Hepatic Porphyria) in Females After Oral Contraceptive Medication. Arch Dermatol Res. 1985;278(1):13-6. PubMed PMID: 4096525.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hereditary uroporphyrinogen-decarboxylase deficiency predisposing porphyria cutanea tarda (chronic hepatic porphyria) in females after oral contraceptive medication. AU - Sixel-Dietrich,F, AU - Doss,M, PY - 1985/1/1/pubmed PY - 1985/1/1/medline PY - 1985/1/1/entrez SP - 13 EP - 6 JF - Archives of dermatological research JO - Arch. Dermatol. Res. VL - 278 IS - 1 N2 - Porphyria cutanea tarda (PCT) was diagnosed in 27 women aged 23-48 years (mean, 35 years) who had been under oral-hormonal-contraceptive medication for 1-18 years, in 3 women under substitutional estrogen treatment in the menopause, and in 2 men aged 65 and 76 years after estrogen treatment of prostatic carcinoma. In all patients, total urinary porphyrin excretion was elevated, with an average uro- and heptacarboxyporphyrin predominance of 88%, thus proving PCT. Of the patients, 84% showed a significant decrease of erythrocyte uroporphyrinogen-decarboxylase (UD; EC 4.1.1.37) activity to approximately 50% of control levels suggesting a hereditary predisposition for the development of a chronic hepatic porphyria. Estrogens and alcohol are capable of reducing hepatic UD activity. Women with hereditary red cell UD deficiency may be regarded as predisposed to PCT when under estrogen intake, especially in combination with the potentiating influence of alcohol and chronic liver disease. Normal erythrocyte UD values in patients with additive alcohol consumption may implicate a stronger inhibitory effect for alcohol on UD, suggesting a merely toxic form of chronic hepatic porphyria. SN - 0340-3696 UR - https://www.unboundmedicine.com/medline/citation/4096525/Hereditary_uroporphyrinogen_decarboxylase_deficiency_predisposing_porphyria_cutanea_tarda__chronic_hepatic_porphyria__in_females_after_oral_contraceptive_medication_ L2 - http://www.diseaseinfosearch.org/result/5879 DB - PRIME DP - Unbound Medicine ER -