Atypical IgA/IgG pemphigus involving the skin, oral mucosa, and colon in a child: a novel variant of IgA pemphigus?
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.
Bruckner
Anna L
AL
Department of Dermatology, University of California, San Francisco, San Francisco, California, USA.
Fitzpatrick
James E
JE
Hashimoto
Takashi
T
Weston
William L
WL
Morelli
Joseph G
JG
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000906:Antibodies / Q000097:blood; D001324:Autoantigens / Q000032:analysis; D001485:Basement Membrane / Q000473:pathology; D002648:Child; D003108:Colonic Diseases / Q000188:drug therapy / Q000276:immunology; D003622:Dapsone / Q000627:therapeutic use; D051184:Desmoglein 3 / Q000032:analysis; D004797:Enzyme-Linked Immunosorbent Assay; D004817:Epidermis / Q000473:pathology; D005455:Fluorescent Antibody Technique; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology*; D007074:Immunoglobulin G / Q000276:immunology*; D008297:Male; D009059:Mouth Diseases / Q000188:drug therapy / Q000276:immunology; D009061:Mouth Mucosa / Q000276:immunology; D010392:Pemphigus / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D000906:Antibodies; D001324:Autoantigens; C071894:DSG3 protein, human; D051184:Desmoglein 3; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D003622:Dapsone; D011241:Prednisone