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

Abstract

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.

Investigation of Antimuscarinic Receptor Autoantibodies in Patients with Acquired Idiopathic Generalized Anhidrosis

“…M3R autoantibodies have been reported to be detected in patients with Sjögren's syndrome [at a rate ranging from 11 to 90% (9, 10)], an autoimmune disease characterized by the infiltration of lymphocytes, mainly T-cells, into the lachrymal and salivary glands, thus resulting in dry eyes and mouth. In patients with Sjögren's syndrome, the infiltration of T-cells may also be found around eccrine glands (11). Tcell infiltration around eccrine glands is also seen in patients with AIGA (12).…”

Acquired Generalized Anhidrosis: Review of the Literature and Report of a Case with Lymphocytic Hidradenitis and Sialadenitis Successfully Treated with Cyclosporine

“…Corticosteroids were administered to 21 cases and a good response was observed in 15 cases, including the 8 cases of IPSF reported by Nakazato et al [15] and the 5 cases with autoimmune disease. SS was found in 5 cases (13.5%) [13,22,23,24,25]. An immunological mechanism against the sweat glands was suggested in 8 cases [22,23,24,25,50,59,60,62], including 4 case of SS [22,23,24,25].…”

“…Various types of anhidrosis have been reported [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44] (table 2). To identify the cause of anhidrosis in a patient, examination from many aspects is required (fig.…”

“…Among cases of anhidrosis, AGA is rare [13,15,22,23,24,25,26,27,44,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65] (table 3). A total of 37 previous cases of AGA with detailed clinical features were identified in the English language literature as far as we could determine.…”

Sjögren’s Syndrome

“…It is unclear whether or not the xerosis is due to infiltrate of the eccrine or sebaceous glands, or dysfunctional response of the residual glands (Tapinos et al, 1999). In many biopsies from SS patients, dryness of the skin has been associated with lymphocytic infiltrates in the eccrine glands (Sais et al, 1998). Similar to SLE patients, antibody and complement fixation is often detected clinically "normal" skin.…”