Track: Case Reports

Topic: Inflammatory Diseases

Abstract:

Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory condition characterized by tissue infiltration of IgG4+ plasma cells and elevated serum IgG4 levels. This condition often affects middle aged males, with most case reports published from Japan. Skin lesions are identified in a minority of cases and rarely occur in isolation without systemic involvement. Cutaneous manifestations can be subdivided into primary eruptions, with massive plasma cell infiltrates, or secondary eruptions, which demonstrate inflammatory skin manifestations of systemic disease. Here, we present a case of a 62-year-old healthy Caucasian female diagnosed with a primary cutaneous form of IgG4-RD without current systemic manifestations. Our patient presented with a linear, erythematous plaque extending from the right superior forehead to the right eyelid. Biopsy demonstrated a dense dermal perivascular and periadnexal infiltrate of lymphocytes, plasma cells, and eosinophils. The plasma cells did not show kappa or lambda light chain restriction. Immunohistochemical staining for IgG4 highlighted nearly all plasma cells. Given the concern for IgG4-RD and possible systemic involvement, the patient underwent labs and imaging which were unremarkable except for elevated serum IgG4 levels. Although our patient presented without signs of systemic involvement, review of the literature shows that cutaneous lesions are most often associated with involvement of the orbit, submandibular, lacrimal, and parotid glands. Our patient has improved with systemic corticosteroids which are an effective treatment option for cutaneous and systemic involvement. This case represents a unique presentation of skin limited disease and an opportunity to review the current literature on this unusual disease process.

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Alexandra Bohmer, MD