| Abstrakt: |
TO THE EDITOR.— The prognoses of patients with mast cell disorders differs according to the exact nature of the disease. Since classification in man is sometimes confusing, the scheme outlined in the Table is proposed. This system may be of value when assessing the prognoses of patients with urticaria pigmentosa (UP) or diffuse cutaneous mastocytosis (DCM).Solitary mastocytoma has the most favorable prognosis, since there is no risk of systemic or malignant transformation. When generalized UP with classical pigmented lesions, or DCM with diffuse cutaneous involvement without pigmented lesions, develops in the neonatal period, there is a risk of systemic involvement and death, especially with more widespread skin involvement.1,2 Children in whom DCM or generalized UP develops after the neonatal period have the good prognosis of adults with DCM, whereas the adult form of generalized UP carries a risk of systemic involvement and malignant transformation.3,4 |
