Revised guideline for the diagnosis and treatment of acquired idiopathic generalized anhidrosis in Japan

Abstract

Acquired idiopathic generalized anhidrosis (AIGA) is characterized by an acquired impairment in total body sweating despite exposure to heat or exercise. Severe cases may result in heatstroke. Most cases of AIGA have been reported in Asia, especially in Japan. However, there is limited information on the epidemiology of this condition, and no diagnostic criteria or appropriate treatment options have been established. This guideline was developed to fill this gap. It contains information on the etiology, diagnosis, evaluation of disease severity and evidencebased recommendations for the treatment of AIGA. Appropriate treatment according to disease severity may relieve the clinical manifestations and emotional distress experienced by patients with AIGA.

Evaluation of the correlation between severity of acquired idiopathic generalized anhidrosis and quality of life scores

“…Based on AIGA assessments and DLQI results, correlation between AIGA severity and DLQI, as well as severity by DLQI subscale, were assessed. To evaluate AIGA severity, physicians assessed the research subjects' condition using hospital test results based on criteria for determining AIGA severity from the AIGA guidelines (Table ) . The criteria for determining AIGA severity include three items: (i) area of anhidrotic/hypohidrotic lesions; (ii) area of painful skin or wheal (cholinergic urticaria may be included); and (iii) symptoms of heat stroke.…”

Successful treatment of refractory dermal pain with etizolam and clonazepam in a patient with acquired idiopathic generalized anhidrosis

“…Infectious endocarditis with multiple cerebral infarctions in a patient with severe atopic dermatitis Dear Editor, Infectious endocarditis (IE) is a rare but serious complication of atopic dermatitis. [1][2][3] Here, we report a case of IE with multiple cerebral infarctions and briefly review related Japanese studies. A 41-year-old woman had severe atopic dermatitis (AD) from early childhood and had been treated by local dermatologists.…”

“…Successful treatment of refractory dermal pain with etizolam and clonazepam in a patient with acquired idiopathic generalized anhidrosis Dear Editor, Acquired idiopathic generalized anhidrosis (AIGA) is a rare disease characterized by widespread anhidrosis/hypohidrosis without obvious cause. 1 It is frequently accompanied by stinging/tingling dermal pain and rash of cholinergic urticaria (CholU) in sweating conditions. 1 Strong chronic pain, rather than heat accumulation by anhidrosis, seriously affects patients' daily lives.…”

“…1 It is frequently accompanied by stinging/tingling dermal pain and rash of cholinergic urticaria (CholU) in sweating conditions. 1 Strong chronic pain, rather than heat accumulation by anhidrosis, seriously affects patients' daily lives. Although corticosteroid-pulse therapy improves anhidrosis in AIGA, 1 the pain is less responsive to treatment.…”

“…1 Strong chronic pain, rather than heat accumulation by anhidrosis, seriously affects patients' daily lives. Although corticosteroid-pulse therapy improves anhidrosis in AIGA, 1 the pain is less responsive to treatment. We herein report a case of AIGA with refractory pain, successfully treated by benzodiazepine receptor agonists such as etizolam and clonazepam with functional and morphological recovery of sweat glands.…”