02021nas a2200373   4500000000100000008004100001260001300042653001000055653002800065653002200093653002100115653002700136653001100163653001000174653001200184653000900196653001700205653002200222653001600244653002000260653001600280100001200296700001600308700001300324700001300337700002800350700001300378700001600391245007800407300001000485490000700495520113100502022001401633       2004                            d  c2004 Feb10aAdult10aDiagnosis, Differential10aDiagnostic Errors10aEndemic Diseases10aGalvanic Skin Response10aHumans10aIndia10aleprosy10aMale10aMedian Nerve10aNeural Conduction10aSural Nerve10aTangier Disease10aUlnar nerve1 aSinha S1 aMahadevan A1 aLokesh L1 aAshraf V1 aChandrasekhar Sagar B K1 aTaly A B1 aShankar S K00aTangier disease--a diagnostic challenge in countries endemic for leprosy.  a301-40 v753 a<p>A case of Tangier disease (TD) is reported from India. The patient had presented with indolent mononeuritis multiplex and trophic ulcers of 16 years duration mimicking Hansen's disease. He received antileprosy treatment for one and a half years. Nerve conduction studies revealed features of demyelinating neuropathy. Biopsies of the sural nerve and skin showed striking vacuolation of Schwann cells and myelin sheaths, and foamy vacuolated fibroblasts, respectively, and no evidence of Hansen's disease. Low levels of apolipoprotein A1 (ApoA1) and cholesterol in the serum and undetectable levels of high density lipoprotein (HDL) and low density lipoprotein (LDL) cholesterol in the blood confirmed the diagnosis of TD. This is the first reported case of TD from a tropical country-India. An attempt to establish a correct diagnosis should be made by demonstrating the histopathological and lipoprotein abnormality to avoid long term medications that are chosen empirically and are unnecessary. The importance of recognising this disease in a country where Hansen's disease is highly endemic cannot be overemphasised.</p>  a0022-3050