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Porphyria cutanea tarda as the most common porphyria.
Acta Dermatovenerol Croat. 2007; 15(4):254-63.AD

Abstract

Porphyrias are an uncommon, heterogeneous group of metabolic diseases caused by inherited or acquired deficiency of specific enzymes in the heme biosynthetic pathway resulting in an excess of porphyrins (red-brown pigments) and their precursors. Accumulation of heme precursors, such as porphyrinogens that are spontaneously oxidized to porphyrins, is responsible for various clinical features. Porphyrias are classified into three groups: erythropoietic, hepatic and hepatoerythropoietic, depending on the primary organ affected. Porphyria cutanea tarda (PCT) is the most common porphyria, characterized by defective uroporphyrinogen III decarboxylase enzyme. There are three types of PCT with typical skin manifestations. Patients with PCT present with skin fragility, erosions, vesicles, bullae, and milia in sun-exposed areas of the skin, sometimes periorbital mottled hyperpigmentation and hypertrichosis, sclerodermoid changes and ulceration. We present the most common pathogenetic, clinical, diagnostic, and therapeutic features of PCT and other types of porphyria.

Authors+Show Affiliations

University Department of Dermatology and Venereology , Sestre milosrdnice University Hospital, Vinogradska cesta 29, HR-10000 Zagreb, Croatia. veckybulat@inet.hrNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

18093456

Citation

Bulat, Vedrana, et al. "Porphyria Cutanea Tarda as the Most Common Porphyria." Acta Dermatovenerologica Croatica : ADC, vol. 15, no. 4, 2007, pp. 254-63.
Bulat V, Lugović L, Situm M, et al. Porphyria cutanea tarda as the most common porphyria. Acta Dermatovenerol Croat. 2007;15(4):254-63.
Bulat, V., Lugović, L., Situm, M., Buljan, M., & Bradić, L. (2007). Porphyria cutanea tarda as the most common porphyria. Acta Dermatovenerologica Croatica : ADC, 15(4), 254-63.
Bulat V, et al. Porphyria Cutanea Tarda as the Most Common Porphyria. Acta Dermatovenerol Croat. 2007;15(4):254-63. PubMed PMID: 18093456.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Porphyria cutanea tarda as the most common porphyria. AU - Bulat,Vedrana, AU - Lugović,Liborija, AU - Situm,Mirna, AU - Buljan,Marija, AU - Bradić,Lada, PY - 2007/12/21/pubmed PY - 2008/5/7/medline PY - 2007/12/21/entrez SP - 254 EP - 63 JF - Acta dermatovenerologica Croatica : ADC JO - Acta Dermatovenerol Croat VL - 15 IS - 4 N2 - Porphyrias are an uncommon, heterogeneous group of metabolic diseases caused by inherited or acquired deficiency of specific enzymes in the heme biosynthetic pathway resulting in an excess of porphyrins (red-brown pigments) and their precursors. Accumulation of heme precursors, such as porphyrinogens that are spontaneously oxidized to porphyrins, is responsible for various clinical features. Porphyrias are classified into three groups: erythropoietic, hepatic and hepatoerythropoietic, depending on the primary organ affected. Porphyria cutanea tarda (PCT) is the most common porphyria, characterized by defective uroporphyrinogen III decarboxylase enzyme. There are three types of PCT with typical skin manifestations. Patients with PCT present with skin fragility, erosions, vesicles, bullae, and milia in sun-exposed areas of the skin, sometimes periorbital mottled hyperpigmentation and hypertrichosis, sclerodermoid changes and ulceration. We present the most common pathogenetic, clinical, diagnostic, and therapeutic features of PCT and other types of porphyria. SN - 1330-027X UR - https://www.unboundmedicine.com/medline/citation/18093456/Porphyria_cutanea_tarda_as_the_most_common_porphyria_ L2 - http://www.diseaseinfosearch.org/result/5879 DB - PRIME DP - Unbound Medicine ER -
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