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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Format: | Article |
Language: | English |
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Wolters Kluwer Medknow Publications
2022-01-01
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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. |
first_indexed | 2024-12-24T01:29:47Z |
format | Article |
id | doaj.art-eda7b932ab97465fb4ad6bc4359de4e3 |
institution | Directory Open Access Journal |
issn | 2319-7250 |
language | English |
last_indexed | 2024-12-24T01:29:47Z |
publishDate | 2022-01-01 |
publisher | Wolters Kluwer Medknow Publications |
record_format | Article |
series | Indian Journal of Paediatric Dermatology |
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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