Idiopathic acquired generalized anhidrosis due to occlusion of proximal coiled ducts

Abstract

Idiopathic acquired generalized anhidrosis is a very rare disease of unknown pathogenesis. We report a 25-year-old man with acquired generalized anhidrosis due to occlusion of the coiled ducts. He did not have sweat secretion over the entire surface of the body, including the palms and soles. Sweat-inducing stimuli provoked tingling pain on the skin. Pilocarpine iontophoresis on the forearm did not induce sweat secretion. Neurological examination did not reveal any abnormality in the central or peripheral nervous system. Skin biopsy showed that the coiled ducts were occluded by an amorphous eosinophilic substance. This amorphous eosinophilic substance was positive with periodic acid-Schiff (PAS) staining and was resistant to digestion by diastase. Electron microscopy demonstrated that the coiled ducts were completely occluded by an amorphous substance. The substance occluding the coiled ducts contained fibrous structures. These findings suggested that the acquired generalized anhidrosis in this patient was caused by occlusion of the coiled ducts by a PASpositive substance probably derived from dark cell granules.

Forkhead transcription factor FoxA1 regulates sweat secretion through Bestrophin 2 anion channel and Na-K-Cl cotransporter 1

“…S1D), and we infer that FoxA1 is part of a selective evolutionary option to implement human body temperature regulation by sweating. Thus far, one patient with idiopathic anhidrosis showed pathophysiology similar to that seen in FoxA1 or Best2 mutant mice (28), and it remains to be determined if variation in either of the genes is implicated in such patients or in differential individual rates of sweating.…”

Virilizing para-adrenocortical adenoma associated with idiopathic-acquired generalized anhidrosis in an adolescent girl

“…IAGA is very rare. Until now, 64 cases of IAGA have been reported, 62 being Japanese (6) . To our knowledge this is the fi rst case report of IAGA in an African-American female.…”

“…Many causes such as Fabry ' s disease, diabetes mellitus, Sjogren ' s syndrome, congenital ectodermal dysplasia and congenital insensitivity to pain with anhidrosis, induce generalized hypohidrosis or anhidrosis (6) . Anhidrosis of any cause induces a severe impairment of daily life as described in our patient.…”

“…The pathogenesis of idiopathic acquired generalized anhidrosis (IAGA) is unknown and no specifi c cause has been established (6) . The impairment is postulated to be in the sweat ducts, secretory portions and/or acetylcholine receptors or sympathetic nerves.…”

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

“…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.…”