Congenital sensory neuropathy as a differential diagnosis for phagocytic immunodeficiency

ABSTRACT

There are few reports about congenital indifference to pain or Hereditary and Sensory Autonomic Neuropathy [HSAN]. Several investigations for pathophysiology of this syndrome have been performed and different classifications about it. In this report we present a case of HSAN type II with general absence of pain and self amputations and leprosy-like damage of extremities which was suspected to be phagocytic immunodeficiency due to past history of repeated ulcer and abscess formation