Paediatric mastocytosis

Paediatric mastocytosis overview

Mastocytosis is a condition where the body makes too many mast cells. Most paediatric cases of mastocytosis are cutaneous mastocytosis (CM), which is generally limited to the skin. Many children with CM (mastocytosis limited to the skin) see reduced symptoms or complete resolution by puberty. However, not all children outgrow the condition. If symptoms persist or become more severe, further evaluation may be needed to check for systemic involvement.^1,2

Mastocytosis is not contagious, and alone does not cause an immune deficiency, and does not make a child immunocompromised. It also does not cause developmental or cognitive impairment. With support from trusted adults to avoid triggers, manage symptoms and take any required medication, most children can attend school, childcare and extracurricular activities just like their peers.

Paediatric mastocytosis symptoms

Symptoms of mastocytosis vary depending on the subtype and can present differently across age groups and patients, even within the same subtype.⁶ In paediatric patients CM usually involves minor skin-related symptoms such as itching, flushing (redness) or a burning sensation. It is generally considered harmless, with no recognised risk of anaphylaxis and a high likelihood of resolving by puberty.³

More broadly, symptoms of mastocytosis can differ significantly between patients regardless of variant.^4,5 Multiple symptoms may appear all at once, which is sometimes described as an attack, flare or episode. These episodes may last from hours to days, and their severity and frequency can vary depending on the patient and the type of mastocytosis.⁶

General symptoms of mastocytosis may include, but are not limited to:^4,5

• Skin – Facial flushing, itching, hives (urticaria pigmentosa), skin lesions, Darier’s sign, dermographism.^4,5,7
• Cardiovascular – Low blood pressure, rapid pulse, heart palpitations and light-headedness or fainting.^4,5,7
• Gastrointestinal tract – Abdominal pain, cramping, ulcers, diarrhoea, nausea and vomiting.^4,5,7
• Respiratory system – Wheezing, shortness of breath and anaphylaxis.^4,5,7
• Neurologic system – Headaches, lightheadedness, fatigue and episodic cognitive function issues (brain fog)*.^4,5
• Haematological – Anaemia.⁸
• Skeletal – Bone pain.^9,10

*Acute episodes of mast cell mediator release and activation may cause transient cognitive impairment when accompanied by hypotension or other significant mediator effects. However, chronic or persistent brain fog is not established to be a direct consequence of excessive mast cell proliferation or activation, and alternative causes should be investigated.

Identifying triggers

While triggers in mastocytosis are sometimes considered more predictable than in mast cell activation syndrome, they can still vary significantly between patients, even among those with the same variant, and may sometimes shift over time.⁶

In children, CM symptoms may be triggered by factors that promote mediator release. While triggers will vary between individuals, commonly reported triggers in children can include physical or mechanical stimuli (such as friction or pressure on the skin from clothing), heat or sudden temperature changes, infections, teething, allergens, and certain medications, including some cough suppressants.¹⁵

Keeping a record of exposures and symptom patterns can be helpful. Over time, this process can reveal patterns that support adjustments to daily routines, medical care and preventative strategies. Understanding which factors contribute to your symptoms can help reduce the risk, frequency and severity of mediator-related flares.

Paediatric mastocytosis management

Most paediatric cases improve over time and do not require long-term medication.^11,12

When needed, sedating or non-sedating H1-antihistamines may help manage skin symptoms. In moderate-to-severe gastrointestinal or systemic cases, mast cell stabilisers such as sodium cromoglycate or leukotriene receptor antagonists may be considered. Topical sodium cromoglicate may assist with skin lesion irritation and itch, corticosteroids may also be utilised to reduce inflammation and the size of skin lesions.

Supportive care

In most cases, limited supportive care is required for children with mastocytosis. However, additional support may be helpful in addressing the emotional and developmental impacts of managing chronic symptoms during early life. In some cases, play therapy or psychological services may be useful. Play therapy can help children process their experiences, build coping skills, and remain engaged in developmentally-appropriate activities, even when adjustments are needed as part of their overall disease management.^13,14

For children who carry adrenaline autoinjectors, supportive care should include age-appropriate education on their safe use, as well as clear language to seek help from adults during an emergency. Families and school staff should know how to recognise symptoms of anaphylaxis and respond effectively. Empowering children with a sense of confidence and understanding around their condition can help reduce anxiety and improve overall safety.¹⁴

Note: Supplemental resources which may support children and their carers through navigation of anaphylaxis risks in early childhood and primary school can be found on the Allergy Aware website. Resources for parents of teenagers and young adults can be found on Allergy250k.

Supportive care throughout school, activity and care environments

While some children with mastocytosis may require little or no accommodation, triggers are individual and periods of increased sensitivity or reactivity can occur. Ensuring that all parties involved in a child’s care are proactively informed about mastocytosis, and that clear collaboration exists between families, carers, educators and activity providers, can promote wellbeing without unnecessary restriction or isolation, while also supporting early recognition of changes in a child’s health so appropriate support can be provided.

The following collaborative approaches may assist families and care providers in supporting a child with mastocytosis.

• Discuss activities and environments that may influence symptoms: Maintain an open and collaborative approach to practical adjustments that may support participation, rather than avoiding activities altogether where reactivity may be a consideration. Where available, families may provide a list of  both well-tolerated activities and those that may contribute to symptoms.Care providers may benefit from checking with families before introducing new or commonly used products or activities. Examples may include dress-up activities, shared costumes or craft smocks, sensory play stations (such as slimes or textured materials), exposure to heat, and the use of sunscreens, soaps or wipes.
• Support two-way sharing about symptom presentation: As both families and school or care providers spend extensive time with children throughout the day, regular communication is valuable and can help detect sometimes subtle changes that may warrant medical support. It may be helpful for care providers to report to families on any new or changing symptoms or behaviours, such as increased fatigue, reduced participation or activity avoidance. Families may also choose to share information about new strategies introduced at home to manage or reduce symptom risk, with similar approaches considered in care or school environments.
• Be aware of medical safety considerations: Ensure up-to-date information is available on the child’s diagnoses, including any secondary conditions such as asthma or food allergies. Where a child has a diagnosis of anaphylaxis, care or school settings should ensure a current ASCIA Action Plan is accessible and that relevant staff have received appropriate training in recognising reactions and administering the child’s prescribed adrenaline. Where asthma is present, sharing asthma management plans and ensuring appropriate training are also importantWhere appropriate for the child’s age and development, ensure the child knows who their key support people are within the care, school or activity setting, so assistance is readily available if symptoms arise.

For more information on MCAS, visit TAMS website.

Citations

1. Review and Updates on Systemic Mastocytosis and Related Entities
2. Childhood Cutaneous Mastocytosis: Revisited
3. Mastocytosis Fact Sheet
4. Position Paper – Diagnosis and Investigation of Mast Cell Activation Disorders and Syndrome
5. Global Classification of Mast Cell Activation Disorders: An ICD-10-CM–Adjusted Proposal of the ECNM-AIM Consortium
6. Mastocytosis
7. Diagnosis and management of patients with mast cell activation syndromes: status 2026
8. Systemic Mastocytosis
9. Endocrine manifestations of systemic mastocytosis in bone
10. Skeletal mastocytosis as a cause of chronic widespread pain
11. Criteria for the Regression of Pediatric Mastocytosis: A Long-Term Follow-Up
12. Mastocytosis in Children
13. Child-Centered Play Therapy and Chronic Illness With Outcome Data: A Retrospective Case Study
14. Effectiveness of play therapy in enhancing psychosocial outcomes in children with chronic illness: A systematic review
15. Molecular Background, Clinical Features and Management of Pediatric Mastocytosis: Status 2021

More mastocytosis information

• Mastocytosis overview
• Mastocytosis symptoms
• Mastocytosis testing
• Mastocytosis triggers
• Mastocytosis treatments
• Mastocytosis supportive care, quality of life and managing side effects
• Mastocytosis FAQ
• Paediatric mastocytosis

Citations

• Mastocytosis citations