Lymphocytic autoimmune hidradenitis, cutaneous leucocytoclastic vasculitis and primary Sjögren's syndrome

We describe a 60-year-old woman with primary Sjögren's syndrome, mixed cryoglobulinaemia and cutaneous leucocytoclastic vasculitis who developed generalized hypohidrosis with a markedly decreased sweating response to pilocarpine chloride. Skin biopsies demonstrated dense peri-eccrine lymphocytic infiltrates in the lower reticular dermis, with glandular atrophy. From previous studies it is evident that although patients with Sjögren's syndrome commonly have skin dryness, a lymphocytic hidradenitis has been documented only in a few cases. The histological findings in this case support the role of autoimmune hidradenitis in the development of hypohidrosis in Sjögren's syndrome.