The Cutaneous Porphyrias - 01/06/14
, Ruwani Katugampola, BM, MRCP, MD b, Alexander V. Anstey, MD, FRCP c, d, Michael N. Badminton, BSc, MBChB, PhD, FRCPath a, eRésumé |
The porphyrias are a group of mainly inherited disorders of heme biosynthesis where accumulation of porphyrins and/or porphyrin precursors gives rise to 2 types of clinical presentation: cutaneous photosensitivity and/or acute neurovisceral attacks. The cutaneous porphyrias present with either bullous skin fragility or nonbullous acute photosensitivity. This review discusses the epidemiology, pathogenesis, clinical presentation, laboratory diagnosis, complications, and current approach to porphyria management. Although focusing mainly on their dermatological aspects, the article also covers the management of acute porphyria, which by virtue of its association with variegate porphyria and hereditary coproporphyria, may become the responsibility of the clinical dermatologist.
Le texte complet de cet article est disponible en PDF.Keywords : Cutaneous porphyria, Erythropoietic protoporphyria, Porphyria cutanea tarda, Variegate porphyria, Congenital erythropoietic porphyria, Hereditary coproporphyria
Plan
| Funding Sources: None. |
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| Conflict of Interest: None. |
Vol 32 - N° 3
P. 369-384 - juillet 2014 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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