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CASE REPORT
Year : 2011  |  Volume : 23  |  Issue : 5  |  Page : 425-428

Clouston syndrome with palmoplantar keratoderma


1 Professor, Department of Conservative Dentistry, People's College of Dental Science and Research Centre, Bhopal Madhya Pradesh, India
2 Assistant Professor, Department of Medical Biochemistry, Chirayu Medical College, Bhopal, Madhya Pradesh, India
3 Postgraduate Student, Department of Oral Medicine and Radiology, People's College of Dental Science and Research Centre, Bhopal, Madhya Pradesh, India
4 Postgraduate Student, Department of Conservative Dentistry, People's College of Dental Science and Research Centre, Bhopal, Madhya Pradesh, India

Correspondence Address:
Manika Singh
Postgraduate Student, Department of Oral Medicine and Radiology, People's College of Dental Sciences and Research Centre, Bhanpur Bypass Road, Bhopal-4620037, Madhya Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.5005/jp-journals-10011-1188

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Clouston syndrome (hidrotic ectodermal dysplasia) is characterized by the clinical triad of nail dystrophy, alopecia and palmoplantar hyperkeratosis. Clouston syndrome is transmitted as an autosomal dominant trait and caused by mutations in the GJB6 gene (13g12), encoding the gap junction protein connexin 30 (C x 30). At present, there is no treatment for the disease and management is purely supportive. The life of span, patients is normal. In this report, a case of 9-year-old boy is presented who had few set of primary dentition, but surprisingly complete absence of permanent dentition which observed radiographically. In this case, anodonfa of permanent dentition was present and no alopecia which is a rare finding.


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