Painless self-mutilation − A case of hereditary sensory autonomic neuropathy type 4

Children with hereditary sensory and autonomic neuropathy (HSAN) Type IV present with loss of pain and temperature sensation and anhidrosis. They may sometimes exhibit aggressive and self-mutilating behavior. We present here the case of a 5-year-old male child who presented with a history of self-mu...

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Main Authors: Prateek Pathak, Mohammad Adil, Syed Suhail Amin, Fatima Tuz Zahra
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2022-01-01
Series:Indian Journal of Paediatric Dermatology
Subjects:
Online Access:http://www.ijpd.in/article.asp?issn=2319-7250;year=2022;volume=23;issue=1;spage=67;epage=70;aulast=Pathak
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author Prateek Pathak
Mohammad Adil
Syed Suhail Amin
Fatima Tuz Zahra
author_facet Prateek Pathak
Mohammad Adil
Syed Suhail Amin
Fatima Tuz Zahra
author_sort Prateek Pathak
collection DOAJ
description Children with hereditary sensory and autonomic neuropathy (HSAN) Type IV present with loss of pain and temperature sensation and anhidrosis. They may sometimes exhibit aggressive and self-mutilating behavior. We present here the case of a 5-year-old male child who presented with a history of self-mutilating behavior leading to ulcers over knees, elbows, toes and occiput, and amputation of index fingers of both hands. The patient had loss of temperature and pain sensation, but touch was normal. There was anhidrosis. Serum uric acid levels were normal. Histamine test was absent, and nerve studies showed decreased conduction velocity. A diagnosis of HSAN Type IV was made. This case is being presented as self-mutilation was the prominent complaint of the parents of the child. We also discuss the differences between HSAN Type IV and Lesch−Nyhan syndrome.
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spelling doaj.art-eda7b932ab97465fb4ad6bc4359de4e32022-12-21T17:22:24ZengWolters Kluwer Medknow PublicationsIndian Journal of Paediatric Dermatology2319-72502022-01-01231677010.4103/ijpd.ijpd_176_20Painless self-mutilation − A case of hereditary sensory autonomic neuropathy type 4Prateek PathakMohammad AdilSyed Suhail AminFatima Tuz ZahraChildren with hereditary sensory and autonomic neuropathy (HSAN) Type IV present with loss of pain and temperature sensation and anhidrosis. They may sometimes exhibit aggressive and self-mutilating behavior. We present here the case of a 5-year-old male child who presented with a history of self-mutilating behavior leading to ulcers over knees, elbows, toes and occiput, and amputation of index fingers of both hands. The patient had loss of temperature and pain sensation, but touch was normal. There was anhidrosis. Serum uric acid levels were normal. Histamine test was absent, and nerve studies showed decreased conduction velocity. A diagnosis of HSAN Type IV was made. This case is being presented as self-mutilation was the prominent complaint of the parents of the child. We also discuss the differences between HSAN Type IV and Lesch−Nyhan syndrome.http://www.ijpd.in/article.asp?issn=2319-7250;year=2022;volume=23;issue=1;spage=67;epage=70;aulast=Pathakcongenital insensitivity to painhereditary sensory and autonomic neuropathyself-mutilation
spellingShingle Prateek Pathak
Mohammad Adil
Syed Suhail Amin
Fatima Tuz Zahra
Painless self-mutilation − A case of hereditary sensory autonomic neuropathy type 4
Indian Journal of Paediatric Dermatology
congenital insensitivity to pain
hereditary sensory and autonomic neuropathy
self-mutilation
title Painless self-mutilation − A case of hereditary sensory autonomic neuropathy type 4
title_full Painless self-mutilation − A case of hereditary sensory autonomic neuropathy type 4
title_fullStr Painless self-mutilation − A case of hereditary sensory autonomic neuropathy type 4
title_full_unstemmed Painless self-mutilation − A case of hereditary sensory autonomic neuropathy type 4
title_short Painless self-mutilation − A case of hereditary sensory autonomic neuropathy type 4
title_sort painless self mutilation a case of hereditary sensory autonomic neuropathy type 4
topic congenital insensitivity to pain
hereditary sensory and autonomic neuropathy
self-mutilation
url http://www.ijpd.in/article.asp?issn=2319-7250;year=2022;volume=23;issue=1;spage=67;epage=70;aulast=Pathak
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AT syedsuhailamin painlessselfmutilationacaseofhereditarysensoryautonomicneuropathytype4
AT fatimatuzzahra painlessselfmutilationacaseofhereditarysensoryautonomicneuropathytype4