Current Issue ( Vol. : 5, Issue: 4, October-December 2018)   

Author: K.Moutaide, H. BayBay, A. Nassiri, S.Elloudi, FZ.Mernissi, Pratyush Jain
Keyword: Mastocytosis, children, Cutaneous.
Page No: 01-05
DOI: 10.30876/JOHR.6.1.2019.01-05
Abstract: Paediatric cutaneous mastocytosis (MC) is a rare and benign disease, affecting mainly children from birth to 2 years of age. Four clinical forms are distinguished: pigment aryurticaria, papulo-nodular MC (or mastocytoma), macular telangiectatic MC and diffuse MC. It is a clonal pathology, KIT dependent, considered favourable evolution, exceptionally fatal. No evolution are predictoris currently identified. Material and methods: Our retrospective study looked at 5 cases of cutaneous mastocytosis collected at the Dermatology Department of Hassan II University Hospital in Fez over a period of 3 years from January 2013 to January 2016. The average age of our patients was 27 months (with extremes ranging from 4 months to 3 years). The sex ratio was 4 (4 boys and 1 girl). For 5 patients, the diagnosis of MC was clinically suspected. The Darrier sign was positive in 2 cases. The histopathological study of cutaneous biopsies confirmed the diagnosis of CM in all cases. The immune histochemical study, done in 3 cases, mounted a diffuse and intense positivity to CD-117 (C-kit). The anatomo-clinical confrontation made it possible to retain the diagnosis of urticaria pigmentosa in 3 patients. In one child, it was a mastocytoma. Finally, the last patient presented at the same time lesions of urticaria pigmentosa in its bullous form associated with papulonodular mastocytosis lesions. No patients had extra-cutaneous manifestations of the disease. Conclusion: Through 5 cases of mastocytosis in children and a review of the literature, wetry to clarify the evolution and we insist on the need to establish standardized criteria for clinical analysis, follow-up recommendations, systematically to know the mutational status of KIT, to define the necessary predictive prognostic factors.Download PDF