Epidermolysis bullosa pruriginosa: A rare presentation with asymptomatic lesions.
Indian J Dermatol Venereol Leprol
;
2013 Mar-Apr; 79(2): 235-237
Artigo
em Inglês
| IMSEAR
| ID: sea-147434
ABSTRACT
Epidermolysis bullosa pruriginosa (EBP) is a subtype of dominant dystrophic epidermolysis bullosa (DDEB) and is clinically characterized by pruritic lichenified plaques or prurigo-like lesions with violaceous linear scarring. Pruritus has always been described as one of the most striking features in EBP. Mutations in COL7A gene, especially in the glycine residue, have been shown to cause this form of DDEB. In this report, we describe a north Indian familial clustering of three cases of EBP, spread across two generations, presenting with hypertrophic lichenoid cutaneous lesions, which were completely asymptomatic. Clinical and histopathological analysis favored the diagnosis of EBP in all three cases. They are being reported for their unusual asymptomatic presentation.
Texto completo:
DisponíveL
Índice:
IMSEAR (Sudeste Asiático)
Assunto principal:
Linhagem
/
Humanos
/
Masculino
/
Pré-Escolar
/
Epidermólise Bolhosa
/
Adulto
/
Diagnóstico Diferencial
/
Doenças Assintomáticas
Tipo de estudo:
Estudo diagnóstico
Idioma:
Inglês
Revista:
Indian J Dermatol Venereol Leprol
Ano de publicação:
2013
Tipo de documento:
Artigo
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