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Urticaria pigmentosa: a review of 67 pediatric cases
J M Azaña1, A Torrelo, I G Mediero
1Department of Pediatric Dermatology, Hospital del Niño Jesús, Madrid, Spain.
Insights
Pediatric urticaria pigmentosa, a mast cell disorder, typically presents in infancy with itching. This study found the condition is generally benign and tends to resolve spontaneously in children.
Area of Science:
- Pediatrics
- Dermatology
- Hematology
Background:
- Mastocytosis is characterized by abnormal mast cell proliferation.
- Urticaria pigmentosa is the most common presentation of cutaneous mastocytosis.
- Pediatric mastocytosis often presents as urticaria pigmentosa.
Purpose of the Study:
- To assess systemic involvement in pediatric patients with urticaria pigmentosa.
- To characterize the clinical presentation and natural history of pediatric urticaria pigmentosa.
- To evaluate the benign nature of childhood mastocytosis.
Main Methods:
- Prospective study of 67 pediatric patients with urticaria pigmentosa.
- Comprehensive assessment including hematologic profiles, serum chemistry, skeletal surveys, and bone marrow aspiration.
- Evaluation of age at onset, symptoms (pruritus), and disease progression.
Main Results:
- Most cases of urticaria pigmentosa presented in the first year of life.
- Pruritus was the predominant symptom.
- Systemic involvement was minimal, with slight anemia in 3 patients, bone lesions in 8, and minor bone marrow changes in 7 patients.
Conclusions:
- Pediatric urticaria pigmentosa is predominantly a benign condition.
- The disease course is typically self-limiting with spontaneous resolution.
- Systemic complications are uncommon in pediatric mastocytosis presenting as urticaria pigmentosa.
Abstract:
Mastocytosis is a disorder of mast cell proliferation that may appear during infancy, childhood, or adulthood. We studied 67 consecutive patients (33 males, 34 females) with urticaria pigmentosa and assessed them fully to determine the presence of systemic involvement. Ages at onset of lesions ranged from birth to 11 years, with most developing in the first year of life. Pruritus was the primary symptom. Hematologic and serum chemistry profile, radiologic skeletal surveys, and bone marrow aspirations were performed. Slight anemia was present in three patients. Radiologic bone lesions were observed in eight. Bone marrow aspirates showed slight changes in six patients, with only an increased number of mast cells in an additional patient. The disease tended to resolve spontaneously. This prospective study emphasizes the benign nature of pediatric urticaria pigmentosa.