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Published on: July 11, 2013
Pediatric cutaneous mastocytosis: a review of 180 patients
Dan Ben-Amitai1, Aryeh Metzker, Herman A Cohen
1Schneider Children's Medical Center of Israel, Petah Tiqva, Israel. danb@clalit.org.il
Insights
Pediatric mastocytosis, primarily cutaneous, often presents as urticaria pigmentosa in children under two. The condition generally has a good prognosis, with most cases being sporadic.
Area of Science:
- Pediatric Dermatology
- Hematology
- Immunology
Background:
- Mastocytosis is a rare disorder involving mast cell infiltration.
- Symptoms can arise from mast cell mediator release.
- Cutaneous mastocytosis affects the skin, with potential for systemic involvement.
Purpose of the Study:
- To investigate the clinical characteristics of childhood cutaneous mastocytosis.
- To analyze patient demographics, lesion types, and disease course.
- To identify prognostic factors in pediatric mastocytosis.
Main Methods:
- Retrospective file review of 180 pediatric patients diagnosed with cutaneous mastocytosis over 20 years.
- Evaluation of gender, age of onset, lesion characteristics, distribution, and associated symptoms.
- Assessment of disease course and familial occurrence.
Main Results:
- 180 pediatric cases identified; male to female ratio 1.5:1.
- Urticaria pigmentosa (65%) and mastocytoma (33%) were the most common variants.
- Lesions predominantly on trunk and limbs; most cases sporadic with good prognosis.
- Familial cases noted in 11% of patients with urticaria pigmentosa.
Conclusions:
- Pediatric mastocytosis is typically sporadic, appearing within the first two years of life, predominantly on the trunk.
- Urticaria pigmentosa is the most frequent clinical presentation.
- The overall prognosis for pediatric mastocytosis is favorable.
Background:
Mastocytosis is a heterogeneous group of diseases characterized by the abnormal infiltration of mast cells in the skin and, sometimes, other organs. Some patients may experience symptoms related to mast cell mediator release.
Objective:
To analyze the clinical features of cutaneous mastocytosis in a large series of children.
Methods:
We conducted a file review of all children clinically diagnosed with cutaneous mastocytosis in our department over the last 20 years. We evaluated gender, age at onset, character and distribution of the lesions, associated symptoms, and course of the disease.
Results:
Altogether, 180 patients with cutaneous mastocytosis were identified. The male to female ratio was 1.5:1. About one-third of patients had a mastocytoma, which was present at birth in over 40% and appeared during the first year of life in most of the remainder. Urticaria pigmentosa was noted in 65% of the patients, presenting at birth in 20% and during the first year in most of the remainder. The majority of lesions was distributed over the trunk and limbs. Different kinds of associated symptoms were noted. Prognosis in general was good. Only 11% of the cases, all urticaria pigmentosa, were familial.
Conclusions:
Most cases of pediatric mastocytosis are sporadic and appear during the first 2 years of life, especially on the trunk. Urticaria pigmentosa is the most frequent variant. The prognosis of pediatric mastocytosis, in general, is good.
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