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Year : 2020  |  Volume : 68  |  Issue : 10  |  Page : 2270-2272

The painless eye: Neurotrophic keratitis in a child suffering from hereditary sensory autonomic neuropathy type IV

1 Department of Pediatric Ophthalmology and Strabismus, Arunodaya Deseret Eye Hospital, Gurgaon, Haryana, India
2 Department of Pediatric Ophthalmology and Strabismus, Sankara Nethralaya, Chennai, Tamil Nadu, India

Correspondence Address:
Dr. Aditya Sethi
Arunodaya Deseret Eye Hospital, Sector 55, Plot NH4, Gurgaon - 122 011, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2101_19

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Hereditary sensory autonomic neuropathy (HSAN) is a group of inherited disorders (total 5 types) that are associated with sensory dysfunction and varying degrees of autonomic dysfunction. HSAN type IV (HSAN-IV) or congenital insensitivity to pain and anhidrosis (CIPA) is a rare genetic disorder inherited in an autosomal recessive manner. We report a case of this very rare genetic disease in a 3-year-old girl child, born to a family in north India with ocular features of neurotrophic keratitis. The diagnosis was made clinically based on the hallmark features of insensitivity to pain and temperature, anhidrosis, self-mutilating behavior with multiple recurrent oral ulcers, nasal bleeds, multiple trophic ulcers over joints, and decreased intellect.

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