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Steatocystoma multiplex: A case report of a rare entity

机译:多发性脂肪囊瘤:一例罕见病例报告

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摘要

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 ( ). 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.
机译:多发性脂肪囊瘤是一种罕见的良性皮肤病,通常表现为许多皮内囊肿,可以散布在身体的任何地方。尽管通常没有症状,但可能会严重毁容。已知一种类型的多发性脂肪囊瘤与编码角蛋白17()的基因的突变的常染色体显性遗传有关。在这种情况下,通常与外胚层组织的其他发育异常同时发生,例如指甲,头发和牙齿。据我们所知,很少有病例报道口腔和颌面部多发性脂肪囊瘤。该报告描述了一例颈部两侧多发性脂肪囊瘤和多个牙齿异常的病例,重点是其临床,放射学和组织病理学特征,以及患者表现出这种情况的家族型的可能性。

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