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.
- The onset is early on and sudden or acute.
- There may be sharp pain or cholinergic urticaria over entire body.
- The serum IgE level may be raised.
- There is a marked response to steroid therapy.
- One case after infectious mononucleosis has been reported [CED 2012;38;156-9].
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.
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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