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- Title
Paediatric mastocytosis: a 12‐year follow‐up cohort.
- Authors
Meni, C.; Georgin‐Lavialle, S.; Le Sachè de Peufeilhoux, L.; Jais, J.P.; Hadj‐Rabia, S.; Bruneau, J.; Fraitag, S.; Hanssens, K.; Dubreuil, P.; Hermine, O.; Bodemer, C.
- Abstract
Summary: This study from Paris, France, followed 53 children with mastocytosis as they grew up. In mastocytosis there are increased numbers of mast cells (best known for their role in allergy) in the body, especially in the skin. In the childhood sub‐types, mast cell numbers are usually increased only in the skin and the condition is generally said to improve around puberty. Symptoms include itch, swelling and blistering in the skin but occasionally, if a lot of chemical messengers are released from huge numbers of mast cells, general ('systemic') symptoms such as flushing, abdominal pain, diarrhoea and headache occur. Most children improved, particularly if they had had mastocytosis for more than 12 years or had reached 16 years of age; in six the condition worsened, and in only three children did the mastocytosis clear completely from the skin. In 11 children the condition had started after two years of age and this was the only factor that predicted what would happen over time: the outcome was worse in this group. Systemic symptoms tended to improve, though they resolved completely in only five children and frequently persisted even if the skin changes improved. Three cases went on to develop more generalised mastocytosis, with involvement of bone in one case, bone and liver in the second case, and gut in the third. Forty‐six children showed a mutation in a gene called KIT, which is already known to be associated with mastocytosis, but there was no relationship between this, the type of mastocytosis, or whether or not the mastocytosis improved, persisted or worsened. Linked Article: Meni et al. Br J Dermatol 2018; 179:925–932
- Subjects
PARIS (France); MAST cell disease; PUBERTY; MAST cells; DIARRHEA; HEADACHE
- Publication
British Journal of Dermatology, 2018, Vol 179, Issue 4, pe178
- ISSN
0007-0963
- Publication type
Article
- DOI
10.1111/bjd.17169