Gingival lesions in a patient with dermochondrocorneal dystrophy (Francoissyndrome)

This report is concerned with gingival manifestations associated with a cas e of dermochondrocorneal dystrophy (DCCD) or Francois syndrome occurring in a 42-year-old woman. Our Department treated this patient for 15 years. Ora l examination of this case revealed a diffuse enlargement and severe inflam mation of the attached gingiva. Systemic findings were similar to those rep orted in the literature for patients with DCCD. Firm papules 3 mm wide, loc alized on the face and on the dorsal surface of the hands, were associated with corneal involvement and progressive and severe articular disorder. Bec ause they recurred after surgical ablation, the gingival lesions became an important problem in the management of the patient. After 10 years of unsuc cessful treatment limited to scaling, oral hygiene control and mouth rinses with 0.2% chlorexidine solution, the patient was submitted to extraction o f the remaining teeth, remodelling osteoplasty and cutaneous graft. An acry lic full denture was inserted. In a follow-up of 7 years, good results for the oral health of the patient were seen.