Periodontal manifestations of von Recklinghausen neuro fibromatosis
A 40-year-old lady reported to the Department of Periodontics M.S.R.D.C from Department of General Surgery M.S.R.M.C with a history of multiple faint rounded densities in the skin, chest pain occasionally, since 8 months and breathlessness since 1 year. Patient's immediate family did no report of neurofibromatosis. Swelling of the right side of the angle of the mandible with limited mouth opening []. There was buccal and cortical bone enlargement extending from anteriorly up to canine and posteriorly up to retro molar region []. Well-defined swelling present in the right palatal region, which was firm in consistency measuring 2 cm × 1 cm approximately and ovoid surface and normal in color []. Areas of oral melanin pigmentation was also seen [].\nFor establishing a diagnosis of neurofibromatosis two or more of the criteria described by Gutman et al. needs to be fulfilled, which includes:\nSix or more café au lait macules: Diameter ≥1.5 cm in post-pubertal individuals ≥0.5 cm in pre-pubertal individuals Two or more neurofibromas of any type, or one plexiform neurofibroma Multiple freckles in the axillary area or groin Optic glioma Two or more Lisch nodules (hamartomas of iris) A distinct osseous lesion, such as Sphenoid dysplasia Thinning of the long bone cortex with or without pseudoarthrosis A first-degree relative (parent, sibling or offspring) who meets the above criteria for NF1.\nIn the present case, diagnostic criteria set by Gutman et al. 1997 were fulfilled. They include:\n6 café au lait spots Specific bone changes, thinning of long bones scoliosis was present.\nEnlargement of the interproximal, marginal, and attached gingiva.\nGeneralized pockets and generalized grade I mobility seen.\nOral prophylaxis and polishing were done and oral hygiene instructions were given with 0.2% chlorhexidine mouthwash.\nBiopsy of the suspected lesion was taken.\nIncisional biopsy from the buccal cortex and the palatal region was carried out under local anesthesia. After 1 week, patient had reduced enlargement of the gums [Figures and ].\nConsidering the intra and extraoral and radiographic findings the following were considered for differential diagnosis:\nMcCune Albright syndrome Multiple lipomatosis Proteus syndrome