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Atypical IgA/IgG Pemphigus Involving the Skin, Oral Mucosa, and Colon in a Child: A Novel Variant of IgA Pemphigus?
Author(s) -
Bruckner Anna L.,
Fitzpatrick James E.,
Hashimoto Takashi,
Weston William L.,
Morelli Joseph G.
Publication year - 2005
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.2005.22408.x
Subject(s) - pemphigus , medicine , immunology , antibody , antigen , paraneoplastic pemphigus , desmoglein 3 , immunoglobulin a , oral mucosa , immune system , pemphigus vulgaris , epitope , autoantibody , pathology , immunoglobulin g
Immune‐mediated blistering diseases are rare in childhood, with the subset of IgA pemphigus being extremely uncommon. A child with a bullous disorder of the oral mucosa, skin, and colon is reported because of the unusual nature of the clinical and laboratory findings. Immunofluorescence studies demonstrated both intercellular epidermal and basement membrane zone deposition of IgA and IgG antibodies, as well as intercellular colonic deposition of IgA antibodies. IgA and IgG ELISA tests were positive for desmoglein 3 as the intercellular epidermal antigen. The generation of multiple antibodies against several potential antigenic targets suggests epitope spreading may play a role in this disease. We believe this represents a novel variant of IgA pemphigus.