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Atypical pemphigus with concomitant IgG and IgA anti-intercellular autoantibodies associated with monoclonal IgA gammopathy.

作者信息

Ohno H, Miyagawa S, Hashimoto T, Nakagawa A, Watanabe K, Nishikawa T, Shirai T

机构信息

Department of Dermatology, Nara Medical University, Japan.

出版信息

Dermatology. 1994;189 Suppl 1:115-6. doi: 10.1159/000246948.

DOI:10.1159/000246948
PMID:8049548
Abstract

A case is reported of a 60-year-old woman with acantholytic vesiculopustular dermatosis and IgA-lambda monoclonal gammopathy. The histopathology of vesiculopustular lesions showed intraepidermal acantholytic and neutrophilic blisters. Direct immunofluorescence revealed intercellular (IC) IgD deposition with concurrent deposits of IgA and C3. Indirect immunofluorescence and immunoblotting studies revealed that the patient had circulating IgG and IgA anti-IC antibodies both of which recognized the 150-kD desmoglein that was pemphigus foliaceus antigen in a bovine desmosome preparation.

摘要

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IgA pemphigus associated with monoclonal gammopathy completely resolved after achievement of complete remission of multiple myeloma with bortezomib, cyclophosphamide and dexamethasone regimen.硼替佐米、环磷酰胺和地塞米松方案治疗多发性骨髓瘤达到完全缓解后,IgA 天疱疮伴单克隆丙种球蛋白病完全消退。
Wien Klin Wochenschr. 2010 May;122(9-10):311-4. doi: 10.1007/s00508-010-1361-x.