Definition:

Acquired idiopathic generalized anhidrosis (AIGA) is a rare condition, characterized by generalized absence of sweating without other autonomic and neurologic dysfunction. Most of the cases reported are Japanese. Various pathomechanisms proposed were idiopathic sudomotor dysfunction, sudomotor neuropathy, sweat gland abnormalities and occlusion of proximal duct. The etiology of AIGA remains unclear because of the rarity of this syndrome. According to the limited studies available, various pathologic abnormalities have been reported. Many patients have associated cholinergic urticaria and raised IgE levels. Immunologic mechanisms are considered to be contributory to the disease. Treatment of AIGA almost always comprises steroid pulse therapy or high-dose oral steroids.