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Hereditary Sensory and Autonomic Neuropathy Type IV (HSAN-IV), also known as Congenital Insensitivity to Pain with Anhidrosis (CIPA), is an extremely rare condition with loss of peripheral unmyelinated and small myelinated nerve fibres, leading to the absence of pain sensation and inability to sweat. Affected patients require careful care in order to prevent debilitating consequences and potential morbidity as a result of recurrent trauma and self-mutilating behaviour. We report a case of 4 years and 10 months old boy who has been diagnosed with HSAN-IV at 6 months of age, which was confirmed by genetic testing. This study was conducted in the largest tertiary medical complex in the Kingdom of Bahrain. He had multiple hospital presentations with various upper and lower extremity injuries requiring a multidisciplinary approach for different management strategies. The diagnosis can be extremely challenging due to an exhausting list of differential diagnosis and limited number of cases provided in the literature.
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Matar et al. (2024) studied this question.
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