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Ig A pemphigus with paraneoplastic pemphigus‐like clinical features showing Ig A antibodies to desmoglein 1/3 and desmocollin 3, and Ig G and Ig A antibodies to the basement membrane zone
Author(s) -
Ueda A.,
Ishii N.,
Temporin K.,
Yamazaki R.,
Murakami F.,
Fukuda S.,
Hamada T.,
Dainichi T.,
Kyoya M.,
Saito C.,
Matsunaga R.,
Kimura S.,
Kawakami T.,
Soma Y.,
Hashimoto T.
Publication year - 2013
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/ced.12050
Subject(s) - paraneoplastic pemphigus , desmoglein , antibody , desmoglein 3 , medicine , pemphigus , basement membrane , immunology , pemphigus vulgaris , desmoglein 1 , pemphigoid , pemphigus foliaceus , prednisolone , direct fluorescent antibody , pathology , bullous pemphigoid , autoantibody , gastroenterology
Summary A 79‐year‐old Japanese woman presented with severe recalcitrant erosions on her oral mucosa, resembling paraneoplastic pemphigus. Using indirect immunofluorescence, we detected Ig A antibodies against the cell surface, and both Ig G and Ig A antibodies against the basement membrane zone. Immunoblotting showed that the Ig G antibodies reacted weakly with bullous pemphigoid 230 and periplakin, whereas the Ig A antibodies did not react with any antigen. Ig A antibodies to both desmoglein (Dsg)1 and Dsg3 were detected by ELISA . Ig A antibodies to desmocollin (Dsc)3 were also detected by using cDNA s for human Dsc1–3 transfected into COS ‐7 cells. Despite treatment with oral prednisolone, high‐dose intravenous immunoglobulin and double‐filtration plasmapheresis, the skin lesions remained active, and the patient died from bronchiolitis obliterans‐like respiratory failure. Despite extensive investigations and postmortem examination, no underlying neoplasms were found. The complex immunopathological findings probably played an important role in the development of the patient's unusual clinical features.