A Case of Chronic Bullous Disease of Childhood That Was Reactive to the Antigen of 120 kDa (LAD-1)
Annals of Dermatology
;
: 209-212, 2011.
Artigo
em Inglês
| WPRIM
| ID: wpr-168730
ABSTRACT
Chronic bullous disease of childhood (CBDC) is an autoimmune blistering disease that is characterized by Immunoglobulin A (IgA) deposits at the basement membrane zone. IgA autoantibodies (aAbs) from the serum of patients with CBDC react with antigens of 97 kDa (LABD97) and 120 kDa (LAD-1), and both of which are fragments of the extracellular domain of bullous pemphigoid 180 (BP180, type XVII collagen). The CBDC sera reacts with the immunodominant NC16a domain of BP180, which is the major region recognized by IgG aAbs in patients with bullous pemphigoid. A five-year-old boy presented with multiple pruritic tense blisters on the umbilical and inguinal areas for six weeks. The direct immunofluorescence of the perilesional area demonstrated linear deposits of IgA at the basement membrane zone. Using immunoblotting and an enzyme linked immunosorbent assay (ELISA), we identified the IgA aAbs reactive to antigens with a molecular weight of 120 kDa (LAD-1), which is a fragment of the extracellular domain of BP180.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Autoanticorpos
/
Membrana Basal
/
Imunoglobulina A
/
Imunoglobulina G
/
Ensaio de Imunoadsorção Enzimática
/
Immunoblotting
/
Estimulação Elétrica Nervosa Transcutânea
/
Vesícula
/
Penfigoide Bolhoso
/
Técnica Direta de Fluorescência para Anticorpo
Limite:
Humanos
Idioma:
Inglês
Revista:
Annals of Dermatology
Ano de publicação:
2011
Tipo de documento:
Artigo
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