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Regression of Urticaria Pigmentosa in Adult Patients With Systemic Mastocytosis
Correlation With Clinical Patterns of Disease
Knut Brockow, MD;
Linda M. Scott, MS, CRNP;
Alexandra S. Worobec, MD;
Arnold Kirshenbaum, MD;
Cem Akin, MD, PhD;
Mary M. Huber, RN;
Dean D. Metcalfe, MD
Arch Dermatol. 2002;138:785-790.
Objective To determine clinical correlates of urticaria pigmentosa (UP) regression
in adult patients with systemic mastocytosis (SM).
Design Cohort study of the natural history of mastocytosis.
Setting National Institutes of Health Clinical Center.
Patients In a study of adult patients referred to the National Institutes of
Health after 1980 and observed for a minimum of 10 years, 12 of 106 adult
patients experienced clearance or fading of UP.
Main Outcome Measures Data from each patient's history and results of physical examination,
laboratory evaluation, and organ biopsy at presentation to the National Institutes
of Health were compared with findings at the patient's most recent visit.
Results In the patients in whom clearance of (n = 5) or a decrease in skin lesions
(n = 7) was noted, UP had persisted from 4 to 34 years (median, 17 years).
Older age was a prognostic feature for regression of UP. Despite improvement
of UP, the 2 patients with SM with an associated hematologic disorder experienced
a deterioration in clinical condition. In the 10 patients with indolent SM,
severity and frequency of symptoms decreased as the UP regressed. However,
bone marrow changes consistent with SM remained.
Conclusions Urticaria pigmentosa regresses in approximately 10% of the older patients
who have SM. In patients with an associated hematologic disorder such as myelodysplasia,
this regression may be accompanied by disease progression. In contrast, regression
of UP in patients with indolent SM parallels a decrease in disease intensity,
although bone marrow findings of indolent SM remain.
From the Laboratory of Allergic Diseases, National Institute of Allergy
and Infectious Diseases (Drs Brockow, Worobec, Kirshenbaum, Akin, and Metcalfe
and Mss Scott and Huber), and Clinical Center Nursing Department (Ms Huber),
National Institutes of Health, Bethesda, Md.
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