Two Cases of Patients with Generalized Anhidrosis and Limb Pain

초록

forty one yr old man has been suffered cholinergic ulticaria. He has been treated with systemic steroid. But this symptom didn't recovered fully. 2 months later, he also noted some change of sense and severe lancinating pain in both legs with loss of sweating. In dermatologic evaluation, face showed dry erythemtous patches, and in both axilla and periumbilical area, erythematous patches noted. Under the impression polyneuropathy, we have studied large and small fibers with nerve conduction study (NCS), sympathetic skin response (SSR), heart rate variability test with deep breathing, Valsalva maneuver (HRT), Quantitative axon reflex test (QSART) and thermoregulatory sweat test (TST). Blood chemistry, serologic evaluations, skeletal surveys were done. The skeletal survey, blood chemistry (glucose, HbA1c, liver, kidney function tests), serologic evaluations, α-galactosidase were normal. IgE elevation was noted without significance. NCS with F waves were normal but SSR showed reduced amplitude in hands and absent response in feet. HRTs were normal. There were generalized loss of sweating on whole body area, and small sweating was noted on both toes and fingers only in TST (Fig. 1). QSART revealed generalized absence of response to acetylcholine, compatible with postganglionic sympathetic dysfunction. For evaluation on the loss of sweat skin biopsy was done on upper back (non-sweating area) and scalp (sweating area). Biopsy result was perivascular and pericrine ductal mild lymphocytic infiltration without significance. He has been diagnosed as non-length dependent pattern, small fiber neuropathy, clinically AIGA (acquired idiopathic generalized anhidrosis). Second patient had tingling & pricking sense on back and limbs, especially when exposed to the sun. He had facial swelling and urticaria for nine months and truncal anhidrosis from 7 months. So he visited a dermatologist and was prescribed with steroid for cholinergic urticaria. A couple of months