Natural evolution in pediatric cutaneous mastocytosis: 10-year follow-up
Justyna Czarny1, Joanna Renke2, Anton Żawrocki3
1Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland.
Insights
Most children with cutaneous mastocytosis (CM) see skin lesion improvement over 10 years, but complete remission before puberty is rare. Symptoms specific to mast cell mediators also show less frequent spontaneous remission in diffuse cutaneous mastocytosis (DCM).
Area of Science:
- Pediatric Hematology
- Dermatology
- Oncology
Background:
- Mastocytosis is a myeloproliferative disorder involving clonal mast cell accumulation.
- It presents heterogeneously, affecting various tissues.
- Understanding long-term outcomes is crucial for patient management.
Purpose of the Study:
- To investigate the 10-year evolution of symptoms in pediatric mastocytosis.
- To determine the long-term clinical course and outcomes.
- To analyze remission rates of skin lesions and mast cell mediator-related symptoms (MC MRSs).
Main Methods:
- A cohort of 55 children with mastocytosis was prospectively monitored for over 10 years.
- Data collected included mast cell mediator-related symptoms (MC MRSs) and clinical disease progression.
- Patients were categorized into maculopapular cutaneous mastocytosis (MPCM) and diffuse cutaneous mastocytosis (DCM).
Main Results:
- Complete remission of skin lesions occurred in 10.3% of patients after 10 years; 17.9% showed no remission.
- Complete remission of MC MRSs was observed in 69.2% of MPCM patients versus 14.3% of DCM patients.
- Partial or major regression of skin lesions was common, but complete regression before puberty was infrequent.
Conclusions:
- Children with cutaneous mastocytosis (CM) often experience regression of skin lesions, though complete remission is rare before puberty.
- Spontaneous remission of MC MRSs is less common in children with DCM compared to MPCM.
- Long-term observation highlights the variable but generally favorable prognosis for skin lesions in pediatric mastocytosis.
Background:
Mastocytosis is a heterogeneous group of myeloproliferative disorders characterized by accumulation of clonal mast cells in various tissues. The aim of this study was to determine the symptoms evolution and outcome after 10 years observation.
Methods:
Fifty-five children with mastocytosis were included in the study group and monitored concerning mast cell mediator-related symptoms (MC MRSs) and clinical course of the disease for a period of ≥10 years.
Results:
Patients presented with a maculopapular cutaneous form of mastocytosis (MPCM) (n = 47) and diffuse cutaneous mastocytosis (DCM) (n = 8). The complete remission (CR) of skin lesions occurred in 10.3% of children after 10 years observation; no remission (NR) was observed in 17.9% children. The CR of skin specific MC MRS occurred in 69.2% children with MPCM and in 14.3% with DCM.
Conclusion:
Most children with cutaneous mastocytosis (CM) eventually experience a major or partial regression of skin lesions, although complete regression before puberty is rare. The spontaneous remission of skin specific MC MRS is less frequent in children with DCM.
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