URTICARIA PIGMENTOSA

Course overview

MASTOCYTOSIS IS A DISORDER CHARACTERIZED BY MAST CELL ACCUMULATION, COMMONLY IN THE SKIN, BONE MARROW, GASTROINTESTINAL TRACT, LIVER, SPLEEN, AND LYMPHATIC TISSUES. THE WORLD HEALTH ORGANIZATION (WHO) DIVIDES CUTANEOUS MASTOCYTOSIS INTO THREE MAIN CATEGORIES. THE FIRST INVOLVES 3 OR FEWER LESIONS CALLED MASTOCYTOMAS. THE SECOND INVOLVES BETWEEN 4 AND 100 LESIONS AND IS REFERRED TO AS URTICARIA PIGMENTOSA (UP). THE THIRD CATEGORY INVOLVES DIFFUSE CUTANEOUS INVOLVEMENT. URTICARIA PIGMENTOSA IS THE MOST COMMON CUTANEOUS MASTOCYTOSIS IN CHILDREN, AND CAN PRESENT IN ADULTS AS WELL. IT IS GENERALLY CONSIDERED TO BE A BENIGN, SELF-RESOLVING CONDITION THAT REMITS IN ADOLESCENCE. UNLIKE THE FORMS OF MASTOCYTOSIS THAT ARE SOLELY SEEN IN ADULTS, INTERNAL ORGAN INVOLVEMENT IS RARE IN URTICARIA PIGMENTOSA. THIS ACTIVITY DESCRIBES THE RISK FACTORS, EVALUATION, AND MANAGEMENT OF URTICARIA PIGMENTOSA AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN ENHANCING CARE DELIVERY FOR AFFECTED PATIENTS.