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[A case of Ehlers-Danlos syndrome].

Shoni shikagaku zasshi. The Japanese journal of... 27(1):208 (1989) PMID 2602708

Ehlers-Danlos syndrome, an inherited connective tissue disorder, is characterized by skin hyperextensibility, joint hypermobility and skin fragility. The disease is at least classified into 10 types, I to X, based on clinical features, biochemical abnormalities of the connective tissue and the mode of inheritance. A case is reported of a 6-year, 8-month-old girl who showed the features of the Ehlers-Danlos syndrome type VII. The findings were as follows: 1) Soft and velvety touch skin, characteristic joint hypermobility, talipes varus, and ventricular septal defects were present. 2) Widely set eyes, slight stabismus, epicanthal folds of the eyes, antimonogoloid slanted eyes, a broad bridge to the nose and low set ears were observed. 3) Gingival bleeding following toothbrushing, Enamel hypoplasia, high arched palate and laxity of the temporomandibular joint were present. 4) In the findings of the maxillary and mandibular dental arches, there was no tendency towards conical teeth in the deciduous dentition, and the width and length of the dental arches were remarkably narrow and short. 5) The roentogenographic findings revealed that there were no minor root deformities, pulpstones and calcification of the pulp. 6) The measurement of the width, length, and depth of the palate through moiré topography revealed that the palate was narrow and deep compared with the control. 7) As a result of cephalometric diagnosis, the nasion appeared to be protruded, therefore maxillary and mandibular seemed to be relatively retracted, and the gonial angle was obtuse. The dental management of patients with Ehlers-Danlos syndrome was discussed and reviewed.