title: Paediatric mastocytosis: a systematic review of 1747 cases.
abstract: Paediatric mastocytosis was previously considered to be a benign and spontaneously regressing disease. However, this evolution is impossible to predict. To clarify the characteristics and course of paediatric mastocytosis, we performed a literature review of 1747 cases published between 1950 and April 2014. Lesions occurred before the age of 2 years in 90% of cases, and presented as urticaria pigmentosa (75% of cases), mastocytoma (20%) or diffuse cutaneous mastocytosis (5%). The male-to-female ratio was 1·4. KIT D816V mutation was detected in 34% of 215 tested patients. Clinical regression (complete or partial) occurred in 67% of cases and stabilization in 27%. However, the outcome was fatal in 2·9% of patients.
authors: Service de Dermatologie de l'Adulte et de l'Enfant, Faculté de Médecine et AP-HP, Hôpital Necker-Enfants Malades, Centre de Référence des Mastocytoses, CEREMAST, Université Paris Descartes, Paris Sorbonne Cité, 149 Rue de Sèvres, 75743, Paris Cedex 15, France.
authors: C
authors: C
authors: Méni
authors: J
authors: J
authors: Bruneau
authors: S
authors: S
authors: Georgin-Lavialle
authors: L
authors: L
authors: Le Saché de Peufeilhoux
authors: G
authors: G
authors: Damaj
authors: S
authors: S
authors: Hadj-Rabia
authors: S
authors: S
authors: Fraitag
authors: P
authors: P
authors: Dubreuil
authors: O
authors: O
authors: Hermine
authors: C
authors: C
authors: Bodemer
mesh_terms: D017668:Age of Onset; D001706:Biopsy / Q000379:methods; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D034701:Mastocytosis, Cutaneous / Q000235:genetics / Q000473:pathology*; D009154:Mutation / Q000235:genetics; D011247:Pregnancy; D011379:Prognosis; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D014582:Urticaria Pigmentosa / Q000209:etiology
chemical_list: D019009:Proto-Oncogene Proteins c-kit