스테로이드로 치료한 후천특발전신무한증 환자의 예후

Abstract

Objective: To elucidate the response to steroid therapy and prognosis in patients with acquired idiopathic generalized anhidrosis (AIGA). Method: We reviewed the medical records of patients seen at a tertiary neurology clinic between March 2017 and July 2019. Inclusion criteria required the diagnosis of widely distributed anhidrosis, affecting at least 50% of the entire body, in the absence of any other autonomic or neurologic dysfunction. The diagnosis and distribution of generalized anhidrosis were confirmed using thermoregulatory sweat test. Results: Six male patients were included with a mean age at onset of 32 years (range, 16~43 years). All patients received corticosteroid therapy with five consecutive days of intravenous 1g methylprednisolone and oral prednisolone. Five patients showed good response to steroid therapy. However, decreased sweating reappeared in more than a half of patients (N = 3/5) with a mean interval of 32 days (standard deviation, 7.5) until recurrence. The differentiated findings in patients with recurrence compared to those without were anhidrosis over a wider part of body on quantitative sudomotor axon reflex test and decreased heart rate variability to deep breathing. Conclusion: Corticosteroid therapy was effective in improving AIGA symptoms. However, recurrence rates seem to be high after treatment discontinuation and prognostic factors need to be confirmed.