Conflict of interest: none declared.
Clinical dermatology ● Concise report
IgA pemphigus with paraneoplastic pemphigus-like clinical features showing IgA antibodies to desmoglein 1/3 and desmocollin 3, and IgG and IgA antibodies to the basement membrane zone
Article first published online: 21 MAR 2013
© The Author(s) CED © 2013 British Association of Dermatologists
Clinical and Experimental Dermatology
Volume 38, Issue 4, pages 370–373, June 2013
How to Cite
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. and Hashimoto, T. (2013), IgA pemphigus with paraneoplastic pemphigus-like clinical features showing IgA antibodies to desmoglein 1/3 and desmocollin 3, and IgG and IgA antibodies to the basement membrane zone. Clinical and Experimental Dermatology, 38: 370–373. doi: 10.1111/ced.12050
- Issue published online: 23 APR 2013
- Article first published online: 21 MAR 2013
- Manuscript Accepted: 25 JUL 2012
A 79-year-old Japanese woman presented with severe recalcitrant erosions on her oral mucosa, resembling paraneoplastic pemphigus. Using indirect immunofluorescence, we detected IgA antibodies against the cell surface, and both IgG and IgA antibodies against the basement membrane zone. Immunoblotting showed that the IgG antibodies reacted weakly with bullous pemphigoid 230 and periplakin, whereas the IgA antibodies did not react with any antigen. IgA antibodies to both desmoglein (Dsg)1 and Dsg3 were detected by ELISA. IgA antibodies to desmocollin (Dsc)3 were also detected by using cDNAs 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.