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Picture of the Month—Diagnosis
Arch Pediatr Adolesc Med. 2008;162(4):384.
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Denouement and Discussion: Urticaria Pigmentosa
Mastocytosis is a disorder characterized by mast cell accumulation in tissue most commonly in the skin, and it may also affect the bone marrow, gastrointestinal tract, skeletal system, liver, spleen, and lymph nodes. A World Health Organization consensus group1 recently divided the disease into 7 forms: cutaneous mastocytosis, indolent systemic mastocytosis, systemic mastocytosis with an associated hematologic non–mast cell lineage, aggressive systemic mastocytosis, mast cell leukemia, mast cell sarcoma, and extracutaneous mastocytoma. Systemic mastocytosis is mostly an adult phenomenon.
CLINICAL FEATURES
Urticaria pigmentosa (UP), the most common type of mastocytosis, is defined by multiple red-brown hyperpigmented macules, papules, or nodules.2 The most recent studies estimate its incidence to be 1 in 150 000.1 In 55% of patients, UP begins before age 2 years, and in 10% of patients it occurs between ages 2 and 15 years. Onset for the remaining 35% of patients occurs between ages 20 and 40 years. Urticaria pigmentosa most . . . [Full Text of this Article] DIAGNOSIS TREATMENT AUTHOR INFORMATION
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Arch Pediatr Adolesc Med. 2008;162(4):383.
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