Mucocutaneous dyskeratosis with periodontal destruction and premature tooth loss.
We report the case of a 16-month-old boy who presented an exuberant erythematous gingival swelling and severe tooth mobility. Radiographic examination confirmed alveolar bone loss, and gingival biopsy showed epithelium containing numerous dyskeratotic cells. Because of feeding difficulties, the enlarged gingival tissue and involved teeth were removed. One year later, similar problems were encountered during the eruption of the deciduous second molars. The patient also exhibited papular skin lesions. Histopathologic features on biopsies of the skin and oral lesions were similar. The oral and cutaneous lesions presented by this patient were similar to those described by From et al. in 1978 in a father and son, reported as dyskeratosis benigna intraepithelialis mucosae et cutis hereditaria--the sole report in the English language. To avoid confusion with hereditary benign intraepithelial dyskeratosis (Witkop-von Sallmann syndrome) we have renamed the condition as mucocutaneous dyskeratosis with periodontal destruction and tooth loss.
Department of Oral Diagnosis, Piracicaba Dental School, University of Campinas, Piracicaba, São Paulo, Brazil. michelleagostini@uol.com.br
Michelle
M
Agostini
Renato
R
Valiati
Jorge Esquiche
JE
León
Mário José
MJ
Romañach
Crispian
C
Scully
Oslei Paes
OP
de Almeida
D002675:Child, Preschool; D005882:Gingival Diseases / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007223:Infant; D015603:Keratinocytes / Q000473:pathology; D008297:Male; D009061:Mouth Mucosa / Q000473:pathology*; D010510:Periodontal Diseases / Q000209:etiology* / Q000473:pathology; D035583:Rare Diseases; D013577:Syndrome; D014078:Tooth Eruption; D014081:Tooth Extraction; D016388:Tooth Loss / Q000150:complications* / Q000473:pathology; D014094:Tooth, Deciduous