Management of Sjögren’s syndrome patients in the department of dermatology

Abstract

Sjögren’s syndrome is a disease characterized by involvement of the exocrine glands，such as the lacrimal glands and salivary glands，and is generally recognized as an autoimmune disorder with various other lesions also besides those of the exocrine glands. Various types of cutaneous lesions can be observed in Sjögren’s syndrome. Among the cutaneous lesions observed in primary Sjögren’s syndrome at our clinic in Dokkyo medical university，pernio，photosensitivity，Raynaud’s cheilitis and angular stomatitis are the most frequently found. The cutaneous lesions that are most strongly associated with Sjögren’s syndrome are annular erythema，hyper-gammaglobulinemic purpura，and an insect-bite-like erythema. Amyloidosis cutis atrophicans or anhidrosis，although rare，are closely associated with Sjögren’s syndrome. Moreover，pernio，photosensitivity，drug eruption，leg ulcers and gangrene are also sometimes seen in cases of Sjögren’s syndrome. Erythema nodosum and urticarial vasculitis are cutaneous lesions of Sjögren’s syndrome associated with fever. Systemic corticosteroid treatment is sometimes needed to control cutaneous ulcers caused by cutaneous vasculitis，gangrene，erythema nodosum，urticarial vasculitis， hypergammaglobulinemic purpura and annular erythema. In regular outpatient clinics，we must pay attention to the extraglandular lesions besides the cutaneous lesions in patients with Sjögren’s syndrome.