Oral manifestations of pachyonychia congenita.
Pachyonychia congenita is a rare genetic disorder characterized mainly by hypertrophy of the nails and hyperkeratosis of the skin and mucosae. Fifty percent of all patients have oral leukokeratosis, which is often painful. The case reported here is of a 41-year-old patient who had white lesions in the form of irregular plaques; these affected multiple regions of the oral mucosa and were sensitive to touch. Histological examination revealed acanthosis, parakeratosis and ballooning of the epithelial cells, consistent with oral leukokeratosis. After therapy including topical steroids and prosthetic rehabilitation, the symptoms resolved.
Bauru School of Dentistry, University of São Paulo, Brazil. paulosss@fob.usp.br
Paulo Sérgio
PS
da Silva Santos
Francesco
F
Mannarino
Rute F
RF
Lellis
Luis Henrique
LH
Osório
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003907:Dexamethasone / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007972:Leukoplakia, Oral / Q000188:drug therapy / Q000473:pathology*; D008310:Malocclusion / Q000628:therapy; D009061:Mouth Mucosa / Q000473:pathology*; D053549:Pachyonychia Congenita / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D012459:Salicylates / Q000627:therapeutic use; D014508:Urea / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D012459:Salicylates; D003907:Dexamethasone; D014508:Urea