Clinical Variants of Cutaneous Mastocytosis in Children: Experience from a Single Center
Narjess Er-rachdy *
Department of Dermatology-Venereology, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco.
Ouissal Essadeq
Department of Dermatology-Venereology, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco.
Laila Benzekri
Department of Dermatology-Venereology, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco.
Nadia Ismaili
Department of Dermatology-Venereology, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco.
*Author to whom correspondence should be addressed.
Abstract
Background: Cutaneous mastocytosis (CM) is a rare pediatric dermatosis characterized by the abnormal proliferation and accumulation of mast cells in the skin. It typically presents in early childhood and is most often confined to the skin, with a generally favorable prognosis.
Material and Methods: We conducted a retrospective study over two years, including children diagnosed with CM. Data included clinical presentation, histological and immunohistochemical findings, and serum tryptase levels.
Results: Nineteen patients were included (11 girls, 8 boys), with a mean age at onset of 2.7 years. Maculopapular CM was the most frequent form (15 cases), followed by diffuse CM (3 cases, including 2 bullous forms) and one mastocytoma. Darier’s sign was positive in all cases. CD117 immunostaining confirmed diagnosis, and serum tryptase was elevated in most patients. No systemic involvement was detected.
Conclusion: CM in children is most often skin-limited and benign. Histology with CD117 and tryptase levels remain key diagnostic tools, especially in atypical or bullous forms.
Keywords: Pediatric mastocytosis, cutaneous mastocytosis, maculopapular mastocytosis, bullous mastocytosis, CD117, darier sign, serum tryptase, skin biopsy, childhood dermatoses