Clinical and behavioral approach of a patient with haberland syndrome: Case report.

Abstract

Introduction: Encephalocranocutaneous lipomatosis (ECCL), Haberland or Fishman syndrome, is a rare, sporadic, congenital neurocutaneous disorder of unknown etiology. The best conclusions about its etiology and pathogenesis are based on an accurate description of the characteristic phenotype of patients with SCLCD. Clinically it is characterized by the classic triad of cutaneous, ocular and CNS involvement, patients present unilateral lesions in tissues of ectodermal and mesodermal origin: skin, eyes and CNS; and whose main marker is the Psiloliparous Nevus. Described by Haberland and Perou in 1970. The rarity and common ignorance of the condition often make diagnosis difficult. The differential diagnosis is made with other neurocutaneous mosaicisms, such as Delleman, Goldenhar or Proteus syndromes, although it can be very difficult due to the great overlap between them. Objective: To describe the clinical and behavioral approach of a patient diagnosed with Haberland syndrome. Case report: A 7 year old female patient attended the Pediatric Dentistry Postgraduate Course for clinical evaluation, presenting a reddish colored, pedunculated tumor lesion with a soft consistency and irregular surface located in the attached gingiva between 84 and 85. Conclusion: It is a congenital disease that requires timely detection and management to avoid sequelae. On the other hand, the methods for modifying behavior in a pediatric dentistry office are focused on avoiding unpleasant behaviors and developing positive attitudes towards oral health.