Mastocytosis refers to rare conditions in which mast cells accumulate in one or more organs, such as the skin and bone marrow.
Systemic mastocytosis (SM) is primarily an adult disease, but it can occur in children, though It is rare and often resolves itself before adolescence.
What is SM?
Systemic mastocytosis (SM) is a rare hematological disease characterized by mast cells that are overactive and accumulate in different parts of the body such as the bone marrow, liver, spleen, gastrointestinal tract and lymph nodes.
In the majority of cases, mastocytosis in children affects only the skin and is known as cutaneous mastocytosis.
Read more about SM testing and diagnosis
Mastocytosis in children can also be referred to as childhood or pediatric mastocytosis when it affects both the skin and the internal organs.
Triggers and symptoms of pediatric mastocytosis
Pediatric mastocytosis can be diagnosed in children from birth to 2 years of age. Less frequently, it can occur in children older than 15, and it is often mistaken for a common rash.
In children with mastocytosis, the triggers and range of symptoms are similar to those of adult SM, but the symptom burden is less severe. Children can also be asymptomatic or present with symptoms that are mostly mild, but this may vary.
The most common symptom for children with mastocytosis is pink or brown spots on the skin. They can be few or many, flat or raised, and are often itchy.
Other less common symptoms include flushing of the skin, fatigue, headache, bone or joint pain, stomach cramps, nausea, vomiting or diarrhea. In rare cases, breathlessness and lightheadedness may occur.
Triggers to be aware of include drinking hot liquids, emotional stress, some medications and food, swimming in cold water, and extreme heat.
Treatment of pediatric mastocytosis
In some cases, no treatment is required. But the main treatments for pediatric mastocytosis are H1 and H2 antihistamines such as hydroxyzine, which block the action of histamines to ease symptoms such as skin itchiness, flushing and hives, as well as gastrointestinal discomfort.
The nonsteroidal pimecrolimus cream is also used to treat skin irritation by preventing the release of histamines and other chemicals from mast cells. The mast cell stabilizer cromolyn sodium also blocks the release of histamines and is used in the treatment of pediatric mastocytosis.
Long-term effects
Most cases of pediatric mastocytosis appear to resolve by puberty. Symptoms of skin irritation, rashes, hives or flushing often fade or disappear completely.
In some cases of cutaneous mastocytosis, the disease can develop into a more systemic form, which will require life-long treatment and symptom management.
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