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[Porphyria cutanea tarda and hepatoerythropoietic porphyria].
Nihon Rinsho. 1995 Jun; 53(6):1427-32.NR

Abstract

Porphyria cutanea tarda (PCT) is induced by an enzyme deficiency of hepatic uroporphyrinogen decarboxylase activity. As the result of this enzyme deficiency, uroporphyrin and coproporphyrin accumulate to the skin and other organs. These porphyrins are excreted into urine because of their easy solubility in water. Exposure to the sunlight of the porphyrin-rich skin induces cutaneous changes. Porphyria cutanea tarda develops mainly in middle-aged males, sometimes in females. Ethylalcohol, estrogenic hormones, and hemodialysis are reported as provocative factors. Hyperpigmentation on exposed areas, skin fragility, vesicles and erosions are common in PCT. Histopathologically, subepidermal bulla is a characteristic finding in PCT. PAS positive materials are also prominent around the small blood vessels in the dermis and dermo-epidermal junctions. Hepatoerythropoietic porphyria (HEP) is usually manifest in early childhood with dark urine and reveals severe cutaneous photosensitivity. This disease is a homozygous form of type II PCT.

Authors+Show Affiliations

Department of Dermatology, Faculty of Medicine, University of the Ryukyus.No affiliation info available

Pub Type(s)

English Abstract
Journal Article
Review

Language

jpn

PubMed ID

7616658

Citation

Nonaka, S, and A Takamiyagi. "[Porphyria Cutanea Tarda and Hepatoerythropoietic Porphyria]." Nihon Rinsho. Japanese Journal of Clinical Medicine, vol. 53, no. 6, 1995, pp. 1427-32.
Nonaka S, Takamiyagi A. [Porphyria cutanea tarda and hepatoerythropoietic porphyria]. Nippon Rinsho. 1995;53(6):1427-32.
Nonaka, S., & Takamiyagi, A. (1995). [Porphyria cutanea tarda and hepatoerythropoietic porphyria]. Nihon Rinsho. Japanese Journal of Clinical Medicine, 53(6), 1427-32.
Nonaka S, Takamiyagi A. [Porphyria Cutanea Tarda and Hepatoerythropoietic Porphyria]. Nippon Rinsho. 1995;53(6):1427-32. PubMed PMID: 7616658.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Porphyria cutanea tarda and hepatoerythropoietic porphyria]. AU - Nonaka,S, AU - Takamiyagi,A, PY - 1995/6/1/pubmed PY - 1995/6/1/medline PY - 1995/6/1/entrez SP - 1427 EP - 32 JF - Nihon rinsho. Japanese journal of clinical medicine JO - Nippon Rinsho VL - 53 IS - 6 N2 - Porphyria cutanea tarda (PCT) is induced by an enzyme deficiency of hepatic uroporphyrinogen decarboxylase activity. As the result of this enzyme deficiency, uroporphyrin and coproporphyrin accumulate to the skin and other organs. These porphyrins are excreted into urine because of their easy solubility in water. Exposure to the sunlight of the porphyrin-rich skin induces cutaneous changes. Porphyria cutanea tarda develops mainly in middle-aged males, sometimes in females. Ethylalcohol, estrogenic hormones, and hemodialysis are reported as provocative factors. Hyperpigmentation on exposed areas, skin fragility, vesicles and erosions are common in PCT. Histopathologically, subepidermal bulla is a characteristic finding in PCT. PAS positive materials are also prominent around the small blood vessels in the dermis and dermo-epidermal junctions. Hepatoerythropoietic porphyria (HEP) is usually manifest in early childhood with dark urine and reveals severe cutaneous photosensitivity. This disease is a homozygous form of type II PCT. SN - 0047-1852 UR - https://www.unboundmedicine.com/medline/citation/7616658/[Porphyria_cutanea_tarda_and_hepatoerythropoietic_porphyria]_ L2 - http://www.diseaseinfosearch.org/result/3340 DB - PRIME DP - Unbound Medicine ER -