Steatocystoma multiplex: A case report of a rare entity
10.5624/isd.2019.49.4.317
- Author:
Nan Young SHIN
1
;
Ju Hee KANG
;
Jo Eun KIM
;
Khantaly SYMKHAMPA
;
Kyung Hoe HUH
;
Won Jin YI
;
Min Suk HEO
;
Sam Sun LEE
;
Soon Chul CHOI
Author Information
1. Department of Oral and Maxillofacial Radiology and Dental Research Institute, School of Dentistry, Seoul National University, Seoul, Korea. hmslsh@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Steatocystoma Multiplex;
Computed Tomography;
Keratin-17
- MeSH:
Clinical Coding;
Hair;
Humans;
Keratin-17;
Neck;
Skin Diseases;
Steatocystoma Multiplex;
Tooth;
Wills
- From:Imaging Science in Dentistry
2019;49(4):317-321
- CountryRepublic of Korea
- Language:English
-
Abstract:
Steatocystoma multiplex is an uncommon benign skin disease, which typically manifests as numerous intradermal cysts that can be scattered anywhere on the body. Although usually asymptomatic, it can be significantly disfiguring. One type of steatocystoma multiplex is known to be associated with the autosomal dominant inheritance of a mutation in the gene coding for keratin 17 (KRT17). In such cases, it is often concurrent with other developmental abnormalities of the ectoderm-derived tissues, such as the nails, hair, and teeth. To the best of our knowledge, few cases have been reported of steatocystoma multiplex of the oral and maxillofacial region. This report describes a case of steatocystoma multiplex of both sides of the neck and multiple dental anomalies, with a focus on its clinical, radiological, and histopathological characteristics, as well as the possibility that the patient exhibited the familial type of this condition.
