Acquired Idiopathic Generalised Anhidrosis: A Clinical Overview of Pathophysiology, Diagnosis and Management

Acquired idiopathic generalised anhidrosis (AIGA) is a rare condition characterised by the complete or partial absence of sweating without any identifiable cause. This review summarised the available literature on the condition and outlined its clinical presentation, underlying mechanisms, diagnostic hurdles, and therapeutic strategies. Three subtypes of AIGA have been described: idiopathic pure sudomotor failure (IPSF), sweat gland failure (SGF), and sudomotor neuropathy (SN). Although, IPSF is the most commonly reported form, the underlying pathophysiology remains poorly understood. Diagnosis is often delayed due to the rarity and unfamiliarity of the condition among clinicians, and it requires careful clinical examination and appropriate functional tests. Treatment is usually based on trial and error, with systemic corticosteroids offering some benefit, particularly in cases suspected to have autoimmune involvement. However, many patients do not experience complete remission, and relapses are common. The lack of standardised diagnostic and treatment protocols highlights the need for greater awareness and further research. This review aims to provide an updated understanding of AIGA while identifying gaps in current knowledge and areas for future investigation.