title: IgG4-related skin lesions in a patient with IgG4-related chronic sclerosing dacryoadenitis and sialoadenitis.
abstract: We describe a 60-year-old man with IgG4-related chronic sclerosing dacryoadenitis and sialoadenitis associated with lymphoplasmacytic and eosinophilic infiltration in erythematous nodules. Physical examination revealed left eye extrusion and small itchy nodules on the scalp and neck. The serum IgG level was 1,570 mg/dL, IgG4 463 mg/dL (29.5%), and IgE 4,554 IU/mL. Lacrimal gland biopsy disclosed prominent infiltrates of IgG4-positive plasma cells and scattered eosinophilic infiltrates with fibrosis, consistent with IgG4-related disease. A skin biopsy of a cutaneous nodule demonstrated that the infiltrated plasma cells around arterioles or venules in the deep dermis and subcutaneous fat tissue were strongly positive for IgG4. Although the swollen lacrimal and parotid gland and itchy subcutaneous erythematous nodules improved rapidly with oral prednisolone at a dose of 20 mg per day, the skin, lacrimal, and parotid lesions deteriorated simultaneously during steroid tapering and improved after increasing the dosage. As skin lesions are easy to biopsy, further study of the skin manifestations of IgG4-related disease will be important in further clarifying the clinical spectrum, pathophysiology and response to therapy of this disorder.
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Japan.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Kakuchi
authors.forename: Kazunori
authors.initials: K
authors.lastname: Yamada
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Suzuki
authors.forename: Naoko
authors.initials: N
authors.lastname: Ito
authors.forename: Kunimasa
authors.initials: K
authors.lastname: Yagi
authors.forename: Masami
authors.initials: M
authors.lastname: Matsumura
authors.forename: Masakazu
authors.initials: M
authors.lastname: Yamagishi
authors.forename: Hisanori
authors.initials: H
authors.lastname: Umehara
authors.forename: Yoh
authors.initials: Y
authors.lastname: Zen
authors.forename: Minoru
authors.initials: M
authors.lastname: Hasegawa
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Takehara
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Kawano
mesh_terms: D001160:Arterioles / Q000276:immunology / Q000473:pathology; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology / Q000473:pathology; D002908:Chronic Disease; D003607:Dacryocystitis / Q000150:complications / Q000276:immunology* / Q000473:pathology*; D003937:Diagnosis, Differential; D004804:Eosinophils / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism*; D008214:Lymphocytes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology; D012598:Sclerosis; D012793:Sialadenitis / Q000150:complications / Q000276:immunology* / Q000473:pathology*; D012867:Skin / Q000098:blood supply / Q000276:immunology* / Q000473:pathology*; D014699:Venules / Q000276:immunology / Q000473:pathology
chemical_list: D007074:Immunoglobulin G