Case report: Vesiculobullous eruption with an atypical IgA deposition pattern in a patient with multiple myeloma-A case report and literature review

Front Immunol. 2023 Feb 10:14:1121380. doi: 10.3389/fimmu.2023.1121380. eCollection 2023.

Abstract

Cutaneous vesiculobullous eruptions associated with multiple myeloma (MM) are rare. Although the development of blisters is mostly driven by amyloid deposits of paraproteins in the skin, autoimmunity may play a role. In this study, we report an unusual case of an MM patient with blisters who presented with both flaccid and tense vesicles and bullae. Direct immunofluorescence revealed IgA autoantibody deposits in the basement membrane zone (BMZ) and intercellular space of the epidermis, which displayed an atypical autoantibody deposition pattern. The patient showed rapid disease progression and died during follow-up. We performed a literature review of autoimmune bullous diseases (AIBDs) associated with MM or its precursors and found 17 previously reported cases. Together with the present case, cutaneous involvement of the skin folds was frequently reported, and mucous membranes were barely affected. IgA pemphigus, with consistent IgA monoclonality, was observed in half of the cases. Five patients displayed atypical autoantibody deposition patterns in the skin; the prognosis of these patients appeared to be poorer than that of other patients. We aim to increase our understanding of AIBDs associated with MM or its precursors.

Keywords: IgA autoantibodies; autoimmune bullous disease; direct immunofluorescence; monoclonal gammopathy; multiple myeloma.

Publication types

  • Review
  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoantibodies
  • Autoimmune Diseases*
  • Blister
  • Humans
  • Immunoglobulin A
  • Multiple Myeloma* / diagnosis
  • Skin Diseases, Vesiculobullous* / diagnosis

Substances

  • Autoantibodies
  • Immunoglobulin A

Grants and funding

This study is supported by the grant from the Natural Science Foundation of China, Sichuan (Grant No. 2022NSFSC1582).