What Is Mastocytosis?
9. Prognosis
The outcome depends on the age of onset of mastocytosis. In urticaria pigmentosa where most patients exhibit symptoms before the age of 2 years old, there is an excellent prognosis and the condition tends to resolve by puberty. It is estimated that the lesions diminish by 10% every year. Life threatening episodes of shock are rarely caused by acute extensive degranulation. Serum baseline tryptase levels can be used to predict the severity of the disease.
Those with adolescent or adult onset urticaria pigmentosa have a poorer outcome as the condition is more likely to be persistent with a higher risk of systemic involvement. The malignant transformation rate for juvenile onset systemic mastocytosis is 7% while in adults it increases to as high as 30%. After the age of 10 years, cutaneous mastocytosis has a poorer prognosis with more likelihood to be persistent, systemic disease, and malignant transformation.
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