Longitudinal Study of Pediatric Urticaria Pigmentosa
Adam Heinze1, Travis J Kuemmet1, Yvonne E Chiu2
1Medical College of Wisconsin, Milwaukee, Wisconsin.
Insights
Urticaria pigmentosa (UP) in children rarely causes severe complications and typically has a minimal impact on quality of life. Most cases do not resolve before adolescence, with earlier resolution linked to specific patient and disease characteristics.
Area of Science:
- Pediatric Dermatology
- Mastocytosis Research
- Clinical Pediatrics
Background:
- Urticaria pigmentosa (UP) is the most common pediatric mastocytosis.
- Systemic signs, symptoms, and triggers are associated with UP.
- The impact of UP on children's quality of life is largely unstudied.
Purpose of the Study:
- Characterize the natural history of pediatric UP.
- Identify triggers and complications associated with UP.
- Determine prognostic indicators and the effect of UP on quality of life.
Main Methods:
- Prospective study of children diagnosed with UP between 2002-2007.
- Follow-up visits every 3 years and yearly telephone interviews.
- Quality of life assessed using the Children's Dermatology Life Quality Index.
Main Results:
- 43 subjects followed for a median of 8.1 years; 26 completed the study.
- At age 12, 6/20 patients had disease resolution; 14 remained active.
- No severe reactions to common triggers; 51% experienced Hymenoptera stings without anaphylaxis.
- Most patients reported mild to no effect on quality of life.
Conclusions:
- Severe complications from known UP triggers are uncommon.
- Pediatric UP typically does not resolve before adolescence.
- UP generally has a minimal impact on children's quality of life.
Background/Objectives:
Urticaria pigmentosa (UP) is the most common form of mastocytosis in children and is associated with systemic signs, symptoms, and triggers. To our knowledge, the effect of UP on children's quality of life has not been studied. The objective of the current study was to characterize the natural history, triggers, and complications of pediatric UP, identify prognostic indicators, and determine its effect on quality of life.
Methods:
Between 2002 and 2007, children with three or more mastocytomas diagnosed by a pediatric dermatologist were recruited during visits at the Children's Hospital of Wisconsin Dermatology Clinic (Milwaukee, WI). Research visits were conducted every 3 years and telephone interviews yearly. The Children's Dermatology Life Quality Index was administered to subjects 4 years of age and older at enrollment. Laboratory test results were collected for subjects younger than 4 years at enrollment. Subjects were followed until UP resolution or study end in August 2015.
Results:
The final cohort size was 43 subjects followed for a median of 8.1 years. Twenty-six subjects were followed through study completion. At age 12 years, 6 patients had disease resolution and 14 remained active. Patients who had disease resolution before age 12 years were more likely to be male and had fewer years of age and smaller lesions, fewer affected areas, and earlier onset. Common medications and anesthetics resulted in no serious reactions. Hymenoptera stings occurred in 51%, with no reports of anaphylaxis. No patient reported a severe effect on quality of life, with most indicating mild to no effect.
Conclusion:
Severe complications are not common with historically identified triggers. Disease does not resolve before adolescence in most children. UP has a minimal effect on quality of life for most children.
