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Background Acquired idiopathic generalised anhidrosis (AIGA) is characterised by the sudden onset of reduced or absent sweating across the body. It is suspected to be an autoimmune process.Case A 25-year-old African male presented to an outpatient clinic with two years of intermittent generalised parasthesiae and heat intolerance. He reported an unpredictable, migratory prickling sensation affecting his face, scalp, torso, arms or legs, which was triggered with heat or exercise and was more noticeable in the winter. He also reported longstanding reduction in his sweating, particularly in the axillae. His neurological examination was normal. He had an elevated IgE (173 IU/L), moderate neutropenia, and mildly elevated LFTs. His ANA was 1:80 with normal ENA and negative anti-dsDNA; he had borderline anti-TPO antibodies. Large fibre nerve conduction studies were unremarkable, but there were impaired sympathetic skin responses in the palms and feet. Autonomic testing showed normal cardiovagal and adrenergic responses, while thermal sweat testing demonstrated sparse sweating on the trunk and extremities, and reduced sweating in the interdigital spaces of the feet. The test was aborted at 40 minutes after a 1 degC elevation in body temperature to 37.5 degC. He had no symptomatic response to antihistamines or gabapentin. Suspecting AIGA, three days of pulse methylprednisolone was arranged. He had complete resolution of symptoms after six weeks, and serial thermal sweat testing at six months showed substantial improvement in the sweating response.Conclusion We report a rare case of AIGA which has demonstrated a promising response to high-dose pulse corticosteroid therapy.