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Acquired Idiopathic Generalized Anhidrosis: An Immunohistopathological Investigation of Peri-glands Infiltrated with Immunoreactive Cells.

© 2015 The Authors. doi: 10.2340/00015555-2034 Journal Compilation © 2015 Acta Dermato-Venereologica. ISSN 0001-5555 Acquired idiopathic generalised anhidrosis (AIGA) is an uncommon entity characterised by an inability to sweat in the absence of any neurological features or sweat gland abnormalities (1). Although its pathogenesis is poorly understood, recent reports suggested the involvement of a T-cell-mediated immune response and the contribution of reduced expression of the acetylcholine receptor and acetylcholinesterase in the eccrine gland (1, 2). In this report, we describe a case of AIGA successfully treated with steroid pulse. Interestingly, immunohistochemical staining revealed the infiltration of IL-17 producing cells, granulysin-bearing cells and pSTAT1 expressing cells around the secretary portion of eccrine glands. Moreover, in parallel with a sweat test by the iodine starch method, decreased reactivity to anti-dermcidin antibody was seen in the secretary portion of eccrine glands before therapy, and was recovered after steroid pulse therapy.