[Clinical aspects of Neurofibromatosis type 1 seen in the Department of Dermatology at University Hospital Antananarivo, Madagascar].

Abstract

Introduction: Neurofibromatosis 1 (NF1) is an inherited disease, in an autosomal dominant manner, with complex multi-system involvements. Prevalence varies from one country to another. However, little is known about neurofibromatosis in African countries, particularly in Madagascar.

Methodology: A descriptive retrospective study from 2014 to 2019 was conducted at the service of dermatology at University Hospital Joseph Raseta Befelatanana in Antananarivo, including all patients with neurofibromatosis according to National Institutes of Health Consensus Conference criteria for whom genealogical investigation could be made.

Results: Among 32 cases of NF1 seen during 6 years, 28 cases were included with a sex ratio M/F of 0.87. The mean age was 24 years ranging from 11 to 54 years. Seventeen patients presented sporadic forms. All patients had "café au lait" spots and cutaneous neurofibromatosis. Three cases presented plexiform neurofibromas which cause significant cosmetic and functional problems by their size and their displayed topography. Fifteen patients had Lisch nodules but no case of optic glioma was identified. Neurological symptoms such as learning difficulties, epilepsy and headache were frequent in our case series. However, access to medical imaging was very limited. Scoliosis was the most common orthopedic complication.

Conclusion: The clinical manifestations of NF1 are extremely variable. Although the possibility of systemic complications seems to be low, patients must be followed up.