Skin lesions demonstrating the typical findings of urticaria pigmentosa/maculopapular cutaneous mastocytosis, diffuse cutaneous mastocytosis or solitary mastocytoma, and typical histologic infiltrates of mast cells in a multifocal or diffuse pattern in an adequate skin biopsy.* In addition, features/criteria sufficient to establish the diagnosis of systemic mastocytosis must be absent.[1-3] There are 3 variants of cutaneous mastocytosis (refer to UpToDate table on the classification of mastocytosis variants).
Slechts 5% van volwassenen heeft de cutane variant van mastocytose. Bij kinderen is dit >90%.
Pathogenese: chronische of epidodische mestcelactivatie leidend tot release van o.a. histamine, tryptase, prostaglandines en cytokines
Mestcelactivatie leidt tot: jeuk, urticaria, bronchoconstrictie, hyppot