The cutaneous porphyrias.
Dermatol Clin
; 32(3): 369-84, ix, 2014 Jul.
Article
en En
| MEDLINE
| ID: mdl-24891059
ABSTRACT
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.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Porfirias
/
Porfirinas
/
Piel
/
Enfermedades de la Piel
/
Luz Solar
/
Protectores Solares
Tipo de estudio:
Diagnostic_studies
Límite:
Humans
Idioma:
En
Revista:
Dermatol Clin
Año:
2014
Tipo del documento:
Article