Cutaneous mastocytosis in Thai children
Srisupalak Singalavanija1, Wanida Limpongsanurak
1Dermatology Unit, Queen Sirikit National Institute of Child Health, College of Medicine, Rangsit University, Bangkok, Thailand. srisupalak@yahoo.com
Insights
Cutaneous mastocytosis in children, primarily urticaria pigmentosa, is a benign skin condition. Most cases appear in infancy and typically resolve without systemic symptoms, showing a favorable prognosis.
Area of Science:
- Pediatric Dermatology
- Hematology
- Oncology
Background:
- Mastocytosis is characterized by mast cell proliferation, predominantly affecting the skin.
- It is more prevalent in infants and children than adults.
Purpose of the Study:
- To define the clinical presentation, therapeutic responses, and outcomes for pediatric cutaneous mastocytosis.
- To evaluate the prognosis of childhood cutaneous mastocytosis.
Main Methods:
- A retrospective analysis of 50 pediatric patients diagnosed with cutaneous mastocytosis between 1994 and 2007.
- Histological confirmation of mast cell infiltration in skin biopsies was required for all cases.
Main Results:
- Urticaria pigmentosa constituted 90% of cases, with 94% of patients developing lesions within the first year of life.
- While most children remained asymptomatic, treatments included antihistamines, mast cell stabilizers, topical corticosteroids, and oral prednisolone, with minimal resolution of skin lesions.
- No family history was reported, and only one patient had an associated germ cell ovarian tumor.
Conclusions:
- Cutaneous mastocytosis in children is generally a benign condition.
- It typically lacks systemic involvement and demonstrates a favorable long-term outlook.
Background:
Mastocytosis is a disorder of mast cells proliferation within various organs, most commonly in the skin. The disease more commonly appears during infancy than adult.
Objective:
To characterize the clinical features, response to therapy and prognosis of cutaneous mastocytosis in children.
Material And Method:
A retrospective study of cutaneous mastocytosis was performed at Queen Sirikit National Institute of Child Health during January 1994 to December 2007.All cases were confirmed by histological diagnosis.
Results:
There were a total of 50 patients. The male to female ratio was 1:1.2. Age at onset of lesions ranged from birth to 7 years. Forty-seven patients (94%) developed skin lesions within the first year of ife. There were 45 cases (90%) of urticaria pigmentosa, 3 cases (6%) of mastocytoma and 2 cases (4%) of diffuse cutaneous mastocytosis. None of the patient had a family history of cutaneous mastocytosis. Most of the children were healthy, except the one who had germ cell ovarian tumor Skin biopsies were performed in all cases and revealed mast cells infiltrate in the dermis. Treatment included oral antihistamine in all cases. Oral mast cell stabilizers were given in 6 patients (12%) and topical corticosteroids in 15 patients (30%). Four patients (8%) were treated with oral prednisolone. The skin lesions resolved only in 1 patient (2%) at age 7.8 years, the others still had skin lesions without systemic symptoms.
Conclusion:
Cutaneous mastocytosis is a benign disease in children without systemic involvement.
