title: IgG4-related disease with cutaneous manifestations treated with rituximab: case report and literature review.
abstract: Immunoglobulin type gamma 4 (Ig)G4-related disease (IgG4-RD) is a relatively recently described clinical entity characterised by elevated levels of serum IgG4 and tissue infiltration of IgG4+ plasma cells in various organ systems. Cutaneous involvement is rare but is becoming increasingly appreciated; typically presenting as erythematous papules and/or nodules that are commonly pruritic. We report a case of IgG4-RD presenting with persistent pruritic papules and unilateral parotid swelling. His serum IgG4 level was elevated and a histological examination of his skin biopsies found a lymphoplasmacytic infiltration with an excess of IgG4+ non-clonal plasma cells. The patient was intolerant of oral prednisolone, however complete resolution of the cutaneous lesions was achieved with the anti-CD20 antibody, rituximab.
authors.affiliation: Department of Dermatology, St Vincent's Hospital, Melbourne, Victoria, Australia.
authors.forename: Chris
authors.initials: C
authors.lastname: Jalilian
authors.forename: H Miles
authors.initials: HM
authors.lastname: Prince
authors.forename: Chris
authors.initials: C
authors.lastname: McCormack
authors.forename: Stephen
authors.initials: S
authors.lastname: Lade
authors.forename: Chan Y
authors.initials: CY
authors.lastname: Cheah
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis / Q000097:blood*; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000737:chemistry / Q000473:pathology; D000069283:Rituximab; D012871:Skin Diseases / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007074:Immunoglobulin G; D007155:Immunologic Factors; D000069283:Rituximab
keywords: IgG4; IgG4-RD; IgG4-related disease; cutaneous IgG4-related disease