Oral ulceration in Stüve-Wiedemann syndrome: a new presentation.
Singh, Kiran Borghol, Khaled Williams, Rhodri McMillan, Kevin
Singh, Kiran
Borghol, Khaled
Williams, Rhodri
McMillan, Kevin
Abstract
Stüve-Wiedemann syndrome (SWS) is a rare, autosomal recessive disorder, causing dysautonomia and multisystem failure. Symptoms include skeletal malformations, restricted joint mobility and desensitisation to pain. Patients with SWS presenting with intraoral lesions are extremely rare and this is probably due to their shortened lifespan. We present a case of a 9-month-old patient who presented to our Oral and Maxillofacial Surgery (OMFS)Unit with a chronic inflamed ulcer affecting the tongue, secondary to trauma from erupting central incisors. We believe that depapillation in conjunction with an increased pain threshold contributed to its development. The patient was successfully treated by extraction of the lower central incisors and intralesional steroid injections under general anaesthetic. This case highlights that patients with SWS can present to the OMFS clinician with oral lesions and that they can be safely managed under general anaesthesia.
MIDER Authors
Citations
Altmetric:
Date
2021-08-03
Type
Article
Other
Other
Subject
Dentistry
Collections
Citation
Singh K, Borghol K, Williams R, McMillan K. Oral ulceration in Stüve-Wiedemann syndrome: a new presentation. BMJ Case Rep. 2021 Aug 3;14(8):e241530. doi: 10.1136/bcr-2020-241530
Journal / Source Title
BMJ Case Reports
DOI
10.1136/bcr-2020-241530
PMID
34344643
Publisher
BMJ Publishing Group
Publisher’s URL
https://casereports.bmj.com/