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Comment & Response
December 2013

IgG4-Related Skin Disease

Author Affiliations
  • 1Department of Dermatology, Mayo Clinic, Rochester, Minnesota
  • 2Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota
JAMA Dermatol. 2013;149(12):1439-1440. doi:10.1001/jamadermatol.2013.7326

To the Editor We read with interest the article recently published in JAMA Dermatology that reported successful treatment of IgG4-related disease (IgG4-RD) of the skin with thalidomide.1 A recently described disease entity, IgG4-RD can affect virtually any organ of the body, and while skin involvement is rare, it may manifest clinically as nonspecific skin papules, plaques, and nodules.2 Ingen-Housz-Oro et al1 rendered a diagnosis of IgG4-RD in the skin of 2 patients with skin nodules based on evaluation of skin biopsy specimens showing dense lymphocytic infiltrates with lymphoid follicles, dermal fibrosis, and a IgG4 to IgG ratio exceeding 75%. Although these patients may indeed have had isolated skin involvement by IgG4-RD, we highlight herein recent advances in the diagnosis of IgG4-RD to emphasize the importance of correlating clinical features, histopathologic evidence, and immunophenotypic data derived from careful interpretation of IgG4 and IgG stains.

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