RABSON MENDENHALL SYNDROME : A CASE REPORT

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J Korean Acad Pediatr Dent. 2004;31(3): 481-485.

권장혁, 박기태

성균관대학교 삼성서울병원 소아치과학교실

Corresponding Author: 박기태 ,

ABSTRACT

Rabson-Mendenhall syndrome(RMS) is first characterized in 1955 by Rabson and Mendenhall. RMS is a rare autosomal recessive variant with insulin resistance. This is due to insulin receptor mutations or other target-cell defects in insulin action. General findings include acanthosis nigricans, hypertrichosis, onychauxis, growth retardation, precocious puberty, genital enlargement, protuberant abdomen and xerotic skin. Characteristic oral and maxillofacial findings include dental dysplasia, coarse facial skin, prognathic jaw and fissured tongue. In this case report, dental characteristics of a 4-year old boy with Rabson-Mendenhall syndrome are described.

Keywords: Rabson Mendenhall syndrome | Insulin receptor | Diabetes mellitus | Early eruption | Macrodontia