Mastocytosis in children and adults is one of the rare diseases; that is, of those that affect less than one for every 2 000 people. It is produced by the accumulation of mast cells, which are cells present in the blood and tissues associated with allergic reactions.
This disease causes more mast cells than normal. Consequently, these accumulate in different parts of the body, especially in the skin or bone marrow. In most cases, mastocytosis in children only affects the skin and does so mildly..
Types of mastocytosis in children
Mastocytosis in children corresponds to two thirds of total cases of this disease. In 97% of cases, the first manifestations appear before the age of 2 years.
This disease occurs in two forms:
Systemic mastocytosis. It comprises several rare and malignant diseases that appear in adulthood. They have to do with an abnormal proliferation of mast cells in different parts of the body.
Cutaneous mastocytosis. It is the most common form of mastocytosis in children. It affects only the skin and does not involve other organs. Its evolution is usually favorable and, in most cases, it disappears before puberty.
Cutaneous mastocytosis It can be of three types: mastocytoma of the skin, maculopapular or diffuse cutaneous. Let's see what each of them is about.
Mastocytoma of the skin
We speak of mastocytoma of the skin, or solitary mastocytoma, when a single lesion appears, in the form of a red or orange lump. In a few cases, there are one or two other injuries. They appear most frequently on the wrists, elbows, and trunk during the first months of life.
The lesion may itch and small blisters or blisters may form on it when scratched or when there are changes in temperature; This is called Darier's sign. They correspond to between 10 and 35% of cases of mastocytosis in children.
Maculo-papular mastocytosis, also called urticaria pigmentosa, it is the most common form of this disease; represents between 70 and 90% of total cases cutaneous mastocytosis. It usually appears in the first months of life and symptoms can vary greatly from case to case.
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In this mode, there are many spots or small bumps all over the skin; lesions come in various shapes and sizes, but they are almost always brown or purplish in color. They can also feature Darier's sign. In 50% of cases it disappears before reaching adolescence; 25% do so when they reach adulthood and 25% become chronic.
Diffuse cutaneous mastocytosis
It is the rarest form of mastocytosis in children, corresponding to between 1 and 3% of cases. Too it is the most difficult modality to treat; therefore, it is very important that it is diagnosed early.
In diffuse cutaneous mastocytosis, the child's skin looks red and thickened, similar to that of elephants. Sometimes, large, tight blisters form on some areas of the skin. The lesions also show Darier's sign, and other symptoms such as diarrhea, bronchospasm, and severe flushing may be present.
Can complications arise?
It is very important that mastocytosis in children is kept under medical supervision strictly. The progression of the lesions and possible additional symptoms must be closely monitored.
Most children affected by this disease do not have complications. In very rare cases anaphylaxis occurs, a serious allergic reaction that can be fatal.
Likewise, and for reasons still unknown, symptoms may be exacerbated when those affected come into contact with one or more of the following substances:
Aspirin or any medicine that contains acetylsalicylic acid.
Contrast substances used in radiological studies.
Non-steroidal anti-inflammatory drugs.
Poison from wasp or bee stings.
Morphine and any of its derivatives.
While mastocytosis in children is a disease that tends to improve and disappear over the years, treatment is primarily aimed at reducing symptoms and avoiding triggers.
There are many occasions in which mastocytosis in children does not require any treatment. Sometimes doctors recommend only a cream with corticosteroids and anthistamines or cromoglycate. If there is diffuse cutaneous mastocytosis, it is likely that more specialized treatment is required, with drugs, phototherapy, and others.
The most important thing is that the child does not scratch or rub the affected area. It is also convenient to avoid sudden changes in temperature, especially at bath time. Both measures are intended to avoid the Darier sign.
Except in specific cases, there are no contraindications to medicines, vaccines or food. Only if there is a fever reaction to a vaccine, it is recommended to use antihistamines prescribed by the doctor.
Except in a few cases, mastocytosis in children has a benign course with mild manifestations. Likewise, it almost always disappears spontaneously, even in complex cases of diffuse cutaneous mastocytosis.
Still, there is a small percentage of children in whom the problem is not resolved by adulthood. Therefore, it is convenient to regularly monitor the disease and put the case in the hands of a specialist.
Mastocytosis: definition, types and diagnosis
Mastocytosis is, actually, a group of rare pathologies that are due to an excessive presence of mast cells in one or more organs. Read more "