マラソンレクチャー
特発性後天性全身性無汗症の診断と治療
キーワード:
acquired idiopathic generalized anhidrosis,
cholinergic urticaria,
regular sweating activities,
heat stroke,
pulse steroid therapy
2025 年 42 巻 4 号 p. 715-719
詳細
2025 年 42 巻 4 号 p. 715-719
Acquired idiopathic generalized anhidrosis (AIGA) is a sweating disorder primarily affecting young to middle–aged males. It causes intense pain and itching upon sweating, significantly impacting daily life. While its pathophysiology remains unclear, corticosteroid therapy is highly effective. However, recurrence occurs in about 50% of cases, requiring long–term follow–up.
AIGA manifests as widespread sweating impairment, often sparing the axillae, face, palms, and soles. Cholinergic pain and urticaria–like eruptions upon sweating lead to heat intolerance and reduced quality of life. Diagnosis is based on clinical criteria, sweat tests, sympathetic skin response, and skin biopsy.
High–dose corticosteroid pulse therapy is the first–line treatment, particularly within the first year. Post–treatment, “sweat training” through exercise, sauna, and warm baths is recommended. Cooling strategies and lifestyle adjustments help manage symptoms, as no definitive pain relief medication exists.
AIGA is often underdiagnosed, leading to treatment delays. Raising awareness and ensuring early intervention can improve outcomes. Further research is needed to clarify the disease mechanism, prevent recurrence, and develop targeted treatments.
