IgG4-related Skin Lesions in a Patient with IgG4-related Chronic Sclerosing Dacryoadenitis and Sialoadenitis

Yasushi Kakuchi; Kazunori Yamada; Yasunori Suzuki; Naoko Ito; Kunimasa Yagi; Masami Matsumura; Masakazu Yamagishi; Hisanori Umehara; Yoh Zen; Minoru Hasegawa; Kazuhiko Takehara; Mitsuhiro Kawano

doi:10.2169/internalmedicine.50.5239

CASE REPORTS

IgG4-related Skin Lesions in a Patient with IgG4-related Chronic Sclerosing Dacryoadenitis and Sialoadenitis

Yasushi Kakuchi, Kazunori Yamada, Yasunori Suzuki, Naoko Ito, Kunimasa Yagi, Masami Matsumura, Masakazu Yamagishi, Hisanori Umehara, Yoh Zen, Minoru Hasegawa, Kazuhiko Takehara, Mitsuhiro Kawano

Author information

Yasushi Kakuchi
Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Japan
Kazunori Yamada
Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Japan
Yasunori Suzuki
Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Japan
Naoko Ito
Division of Cardiology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Japan
Kunimasa Yagi
Division of Cardiology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Japan
Masami Matsumura
Research Center for Medical Education, Kanazawa University Graduate School of Medicine, Japan
Masakazu Yamagishi
Division of Cardiology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Japan
Hisanori Umehara
Hematology and Immunology, Kanazawa Medical University, Japan
Yoh Zen
Institute of Liver Studies, King's College Hospital, UK
Minoru Hasegawa
Department of Dermatology, Kanazawa University Graduate School of Medical Science, Japan
Kazuhiko Takehara
Department of Dermatology, Kanazawa University Graduate School of Medical Science, Japan
Mitsuhiro Kawano
Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Japan

Keywords: IgG4-related disease, cutaneous lymphoid infiltrate, IgG4-related chronic sclerosing dacryoadenitis and sialoadenitis

JOURNAL OPEN ACCESS

2011 Volume 50 Issue 14 Pages 1465-1469

DOI https://doi.org/10.2169/internalmedicine.50.5239

Details

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.

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