Digital Amputation by Congenital Insensitivity to Pain with Anhidrosis

An 18-month-old boy was referred to the pediatric neurology clinic for evaluation of finger ulcers. He was born to nonconsanguineous parents with uncomplicated delivery. Detailed history revealed early dislocation of his first teeth, easy fatigability in summer, and autistic trait. On examination, the first and second digits of both hands were amputated distally (Figure). His palms were warm and dry. Thermal sweating test revealed complete anhidrosis. Palmar sympathetic skin response was not seen.

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