Abstract: IgG4-related sclerosing diseases are newly recognized, which are considered to be related to autoimmunity, including cellular and humoral immune abnormity. IgG4 levels in serum are higher than normal. Histopathological findings include fibrosis with significant inflammatory cells, mainly IgG4(+) plasma cells. Lesions can involve many internal organs. Skin lesions are mostly found in head and neck, presenting with erythema, patches or subcutaneous nodules accompanying itching. Glucocorticoid is effective in the treatment of IgG4-related skin diseases.

Key words: IgG, autoimmune disease, sclerosing disease, skin