Background: Acquired idiopathic generalized anhidrosis (AIGA) is a rarely encountered clinical syndrome. Sixty-five cases have been reported and 62 of them are Japanese. AIGA was further classified into 3 subgroups with idiopathic pure sudomotor failure being the most common. However, it is burdensome to diagnose AIGA and identify its subgroups. Some of the tools used to diagnose AIGA such as the quantitative sudomotor axon reflex test and microneurography are not generally available.
Case summary: We report the first Chinese patient with AIGA and review the literature to develop a flowchart for the diagnosis and identification of subgroups of AIGA.
Conclusion: We conclude that skin biopsy is crucial for the identification of subgroups of AIGA.