ACQUIRED IDIOPATHIC GENERALIZED ANHIDROSIS

By Gary M. White, MD


Acquired idiopathic generalized anhidrosis (AIGA) is a rare disorder in which systemic anhidrosis occurs in the absence of any causative skin, metabolic, or neurological disorder.

Clinical

Patients have a diffuse inability to sweat. AIGA can lead to itching, cholinergic urticaria, discomfort, hyperthermia, and even heat stroke. Of note, hypohidrosis may be seen in Sjögren's syndrome and Fabry disease.

Treatment

High doses of systemic corticosteroids have been used successfully in several patients [J Dermatol Case Rep. 2014 Dec 31; 8(4): 120–121].

Nine patients with AIGA were treated with pulse steroid therapy with excellent results [British Journal of Dermatology, 172: 537–538]. One, two, or three courses of methylprednisolone pulse therapy (3 days, 1000 mg/day) were given. Sweating improved in all patients after therapy. Adverse effects and recurrence of symptoms were not observed for > 1 year after therapy.

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