Steatocystoma multiplex: A case report of a rare entity
신난영, 강주희, 김조은, Symkhampa Khantaly, 허경회, 이원진, 허민석, 이삼선, 최순철,
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신난영 ( Shin Nan-Young ) - Seoul National University School of Dentistry Department of Oral and Maxillofacial Radiology
강주희 ( Kang Ju-Hee ) - Seoul National University Dental Hospital Department of Oral and Maxillofacial Radiology
김조은 ( Kim Jo-Eun ) - Seoul National University Dental Hospital Department of Oral and Maxillofacial Radiology
( Symkhampa Khantaly ) - Seoul National University School of Dentistry Department of Oral and Maxillofacial Radiology
허경회 ( Huh Kyung-Hoe ) - Seoul National University School of Dentistry Department of Oral and Maxillofacial Radiology
이원진 ( Yi Won-Jin ) - Seoul National University School of Dentistry Department of Oral and Maxillofacial Radiology
허민석 ( Heo Min-Suk ) - Seoul National University School of Dentistry Department of Oral and Maxillofacial Radiology
이삼선 ( Lee Sam-Sun ) - Seoul National University School of Dentistry Department of Oral and Maxillofacial Radiology
최순철 ( Choi Soon-Chul ) - Seoul National University School of Dentistry Department of Oral and Maxillofacial Radiology
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.
키워드
Steatocystoma Multiplex; Computed Tomography; Keratin-17
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