title: Pachyonychia congenita. A clinical, histological and microradiographic study with special reference to oral manifestations.
abstract: This paper rresents a clinical, histological and microradiographic study of three patients with pachyonychia congenita with special reference to oral manifestations. The patients, who are relatives, exhibited thickening of finger- and toe-nails, follicular keratosis, palmoplantar keratosis and hyperhidrosis, oral leukokeratosis, and natal teeth. It is stated in the discussion that natal teeth and oral leukokeratosis may constitute the earliest clinical manifestations of pachyonychia congenita and that they appear to accur earlier than nail lesions. When there is a hereditary disposition for pachyonychia congenita, it is important to inspect the oral cavity at an early stage.
mesh_terms: D000328:Adult; D002675:Child, Preschool; D004476:Ectodermal Dysplasia / Q000000981:diagnostic imaging / Q000473:pathology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008852:Microradiography; D009059:Mouth Diseases / Q000000981:diagnostic imaging / Q000473:pathology*; D009264:Nails, Malformed / Q000000981:diagnostic imaging / Q000473:pathology*; D010375:Pedigree