Management of a pemphigus with IgA and IgG antibodies and coexistent lung cancer

Dermatol Ther. 2014 Jul-Aug;27(4):236-9. doi: 10.1111/dth.12126. Epub 2014 Apr 22.

Abstract

Immunoglobulin A (IgA) pemphigus is a clinically distinct variant of pemphigus characterized by intercellular IgA deposition in the epidermis. Recently, an IgA/Immunoglobulin G (IgG) subset of pemphigus with IgA and IgG anti-keratinocyte cell surface antibodies has been described. Both IgA and IgA/IgG pemphigus have been associated with internal malignancies. Above all, monoclonal IgA gammopathy in patients with IgA pemphigus has been reported, and lung cancers in association with IgA/IgG pemphigus have been described. IgA pemphigus can be successfully treated with dapsone, whereas therapeutic management of IgG/IgA pemphigus is not well established yet. We report a rare case of a patient, who developed atypical pemphigus with both IgA and IgG autoantibodies and an underlying lung cancer, successfully treated with corticosteroids and dapsone.

Keywords: IgA/IgG pemphigus; dapsone; lung cancer.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Autoantibodies / immunology
  • Dapsone / administration & dosage
  • Dapsone / therapeutic use
  • Drug Therapy, Combination
  • Glucocorticoids / administration & dosage
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunoglobulin A / immunology*
  • Immunoglobulin G / immunology*
  • Lung Neoplasms / pathology*
  • Male
  • Pemphigus / drug therapy*
  • Pemphigus / immunology
  • Treatment Outcome

Substances

  • Autoantibodies
  • Glucocorticoids
  • Immunoglobulin A
  • Immunoglobulin G
  • Dapsone