Acquired palmoplantar keratoderma and immunobullous disease associated with antibodies to desmocollin 3.
Br J Dermatol
; 157(1): 168-73, 2007 Jul.
Article
em En
| MEDLINE
| ID: mdl-17578440
ABSTRACT
We present a case of immunobullous disease with an impressive acquired palmoplantar keratoderma (PPK) and unique antigenicity. The palms of the patient showed hyperkeratotic ridges with a tripe pattern that decreased with the amelioration of the immunobullous condition. The histopathology of perilesional skin (blister) demonstrated eosinophilic spongiosis and suprabasal blistering as in pemphigus vulgaris. In palmar skin, acantholysis, intraepidermal pustules, papillomatosis and marked hyperkeratosis were observed. Direct and indirect immunofluorescence displayed intraepidermal intercellular IgG staining as well as linear IgG staining along the epidermal basement membrane zone. Immunochemical assays revealed IgG antibodies to the desmosomal protein desmocollin 3 and to the hemidesmosomal proteins BP230 and LAD-1. Affinity-purified antidesmocollin 3 serum IgG bound to monkey oesophagus in the typical pemphigus pattern. Desmocollins are transmembrane proteins of the desmosome. Desmosome diseases may cause hereditary PPK. In our patient with acquired PPK, we hypothesize that the antibodies to desmocollin 3 were, apart from their role in eliciting the pemphigus-like blistering disease, also implicated in the pathogenesis of the PPK.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Complexo Vitamínico B
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Pênfigo
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Niacinamida
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Ceratodermia Palmar e Plantar
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Desmocolinas
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Anticorpos
Tipo de estudo:
Risk_factors_studies
Limite:
Humans
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Male
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Middle aged
Idioma:
En
Ano de publicação:
2007
Tipo de documento:
Article