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Cafe-au-lait spots and pigmented freckling are found in most of patients with neurofibromatosis 1 (NF1). Although many modalities have been used for treating the pigmented lesions, the response to treatment has been variable. Therefore, we performed the treatment of pigmented lesions with NF1 by intense pulsed-radio frequency (IPL-RF) in combination with topical application of vitamin D(3) ointment. Eight patients were treated in this study and the improvement was moderate to good in six cases (75%) although the response was relatively mild. Thus, results from our study indicate that IPL-RF irradiation in combination with topical application of vitamin D(3) ointment would be useful as new modalities, especially for treatment of numerous small pigmented lesions in patients with NF1. Although further studies with large groups of patients should be performed for a better conclusion, it could improve quality of life with NF1 patients who are concerned with serious cosmetic and social problems.

Background: An 8-year old girl was referred for diagnosis and treatment to the Department of Periodontology Oral Surgery of the University Hospital of Liège with an unusual clinical situation: a major, non-inflammatory, diffuse but unilateral enlargement of the interproximal, marginal and attached gingiva around all teeth of the right side of both the upper maxilla and mandible, whereas the alveolar process of the left side of upper and lower arches appeared strictly normal., Method: The clinical examination showed delayed eruption of some permanent teeth in the 1st and 4th quadrants. Except for its asymmetric occurrence, this gingival enlargement strongly resembled phenytoin-induced enlargement or gingival fibromatosis. This unilateral expression was evocative of a vascular or neurologic pathology. Several large "café-au-lait" spots were found disseminated on the body. Several selective surgical removals of thick gingival caps impairing the eruption of some permanent teeth were performed, and the removed tissues were histologically analyzed., Results: Because of the presence of the large "café-au-lait" spots, a clinical diagnosis of Von Recklinghausen's disease was given and later confirmed several times by the histological analysis of the gingival biopsies. Now, 6 years later, this gingival enlargement due to the development of intra-gingival neurofibromas is stable and all permanent teeth have had a normal eruption, but alveolar bone growth has been partly impaired by the presence of the tumor., Conclusions: The present case of unilateral diffuse hyperplasia is a unique clinical expression of neurofibromatosis type I, a slowly evolving neurodermic dysplasia.