To advance the treatment of Acquired Idiopathic Generalized Anhidrosis (AIGA).
AIGA is suspected to be an autoimmune disorder. Glucocorticoids are the most reported treatment, but the optimal dosing is not known. High-dose intravenous methylprednisolone is often used, which requires hospital admission or multiple visits to an infusion clinic.
We present two cases of AIGA treated in the outpatient setting with oral prednisone.
Case 1: a 31-year-old man presented with 10 months of diffuse anhidrosis, initially subacute progressive and now static. Autonomic testing confirmed isolated post-ganglionic sudomotor reflex failure. Skin biopsy showed perivascular and periadnexal inflammation. Lab testing was negative for other autoimmune diseases. At 12 months from symptom onset, he initiated oral prednisone at 60mg daily. After one week of this dose, the dose was reduced by 20mg each week.
Case 2: a 22-year-old man with 2 years of progressive burning paresthesia, heat-induced erythema, inducible urticaria, and anhidrosis. He had no appreciable sensory loss on examination. Autonomic testing confirmed isolated post-ganglionic sudomotor reflex failure. Skin biopsy showed perivascular and periadnexal inflammation. Lab testing was negative for other autoimmune diseases. He was placed on Prednisone 60mg daily for 30 days followed by a two-week taper.
Two months after treatment initiation, both patients reported near complete return of sweat function. Of note, case 1 did report some mild return of sweat function just prior to prednisone initiation, however, his sweat function rapidly improved after just one week of prednisone.