Immunoglobulin G4-related disease and its skin manifestations.

Immunoglobulin G4-related disease and its skin manifestations.

J Dermatol. 2017 Mar;44(3):288-296

Authors: Takayama R, Ueno T, Saeki H

Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory condition characterized by tissue infiltration with IgG4(+) plasma cells and an elevated serum IgG4 level. It usually occurs in middle-aged or older patients, with male predominance, and can involve multiple organs synchronously or metachronously. Patients with IgG4-RD generally respond to steroid therapy. IgG4-related skin lesions are typically erythematous and itchy plaques, subcutaneous nodules, or papules like prurigo nodularis located on the head and neck areas. Histologically, lymphoplasmacytic infiltrates with abundant IgG4+ plasma cells are seen in the dermis and/or subcutaneous tissue. The skin condition of IgG4-RD does not necessarily exhibit infiltration of IgG4(+) plasma cells, which is a prerequisite for IgG4-related skin disease, but such skin lesions without infiltration by IgG4(+) plasma cells can be included in the skin manifestations of IgG4-RD.

PMID: 28256769 [PubMed - in process]

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