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IgG4 ‐related disease with cutaneous manifestations treated with rituximab: Case report and literature review
Author(s) -
Jalilian Chris,
Prince H Miles,
McCormack Chris,
Lade Stephen,
Cheah Chan Y
Publication year - 2014
Publication title -
australasian journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.67
H-Index - 53
eISSN - 1440-0960
pISSN - 0004-8380
DOI - 10.1111/ajd.12100
Subject(s) - medicine , igg4 related disease , rituximab , prednisolone , cd20 , pathology , infiltration (hvac) , antibody , disease , dermatology , immunology , lymphoma , gastroenterology , physics , thermodynamics
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.