Recalcitrant facial pemphigus vulgaris: correlation of skin lesions with the ratio of antidesmoglein antibodies 1 and 3.
Clin Exp Dermatol
; 36(3): 284-7, 2011 Apr.
Article
em En
| MEDLINE
| ID: mdl-21418270
ABSTRACT
Pemphigus vulgaris (PV) is an autoimmune bullous disease characterized by autoantibodies against desmogleins. We report a case of recalcitrant PV, which progressed from the mucosal to the mucocutaneous type, with a corresponding increase in anti-desmoglein (Dsg)1 and decrease in anti-Dsg3 antibody titres. Thus, the clinical features seemed to correlate with the ratio of anti-Dsg1 and 3. The patient also had anti-Dsg4 antibodies, which might be related to the nonscarring diffuse hair loss and marked facial involvement she also had. The patient did not respond to treatment with systemic steroid, ciclosporin, azathioprine, cyclophosphamide or double filtration plasmapheresis, and eventually died from fulminant thrombotic thrombocytopenic purpura of unknown cause.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Autoanticorpos
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Pênfigo
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Desmogleína 1
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Desmogleína 3
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Dermatoses Faciais
Limite:
Female
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Humans
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Middle aged
Idioma:
En
Ano de publicação:
2011
Tipo de documento:
Article