A form of mastocytosis is referred to as urticaria pigmentosa. It appears mainly in young children.
What is urticaria pigmentosa?
Urticaria pigmentosa is the most common form of mastocytosis. Mastocytosis is a rare disease in which mast cells accumulate in the skin and internal organs. In medicine, urticaria pigmentosa, which is one of the benign diseases, is also known as cutaneous mastocytosis. For hyperostosis 101, please visit photionary.com.
It differs from systemic mastocytosis in that mast cells accumulate exclusively in the skin. In the systemic form, on the other hand, organs such as the liver, spleen and intestines are also affected. In addition, the systemic form mainly affects adults, while urticaria pigmentosa is primarily found in children.
The disease is not contagious. White blood cells are called mast cells. They can be found in everyone and contain substances such as heparin, serotonin, histamine and various enzymes that are among the inflammatory mediators. The mast cells perform the function of raising an alarm in the body when they come into contact with pathogens.
In doing so, they release substances such as histamine, which is the prelude to inflammation. This leads to swelling of the skin, dilation of the skin vessels, redness and itching. Wheals also develop. Urticaria pigmentosa usually appears for the first time during the first 24 months of life.
Urticaria pigmentosa, or cutaneous mastocytosis, results from the accumulation of mast cells in the skin. However, it has not yet been clarified what causes this accumulation. Some doctors suspect the reason for the disease to be a genetic defect that triggers uncontrolled growth of mast cells.
This is a point mutation of the c-KIT gene. C-KIT stands for the receptor tyrosine kinase KIT. However, the c-KIT mutation is not the only trigger, since no changes in the c-KIT gene could be detected in many affected children. The triggers for the release of the mast cell components vary from patient to patient and depend on the form of the mastocytosis.
In the case of urticaria pigmentosa, these can be, for example, cold, heat, friction, infections or stress. Food additives, hot spices, allergies, insecticides, alcohol and the intake of certain medications such as acetylsalicylic acid, quinine, codeine or narcotics are also possible. Basically, urticaria pigmentosa is a pseudoallergy that triggers the symptoms.
Symptoms, Ailments & Signs
The symptoms of urticaria pigmentosa vary greatly from person to person. So they can be both slight and strong. In most cases, the condition affects children, who present with reddish-brown patches of skin that usually appear on either the trunk or thighs.
Furthermore, redness and swelling on the skin and blisters are noted. Itching is often noticeable when rubbing the spots. Wheal formation occurs later. Severe cases of cutaneous mastocytosis present with headache, diarrhea, vomiting, and difficulty breathing. Even unconsciousness is possible. However, systemic mastocytosis, which also affects the internal organs, occurs in only about 10 percent of all patients.
Diagnosis & course of disease
If there is a suspicion of urticaria pigmentosa, the doctor first interviews the patient. It is also based on the typical mastocytosis symptoms such as spotting, wheals and itching. In order to be able to make a reliable diagnosis, the doctor carries out a Darier test. It involves scratching or rubbing an affected area of skin.
If the area reddens or itch after a few seconds, the Darier test is classified as positive and the suspicion of cutaneous mastocytosis has been confirmed. It is also possible to take a skin sample, which will be examined in a pathological laboratory. A blood test can also be carried out to determine the triptase level.
In the case of cutaneous mastocytosis, the triptase level is usually normal, while it is higher in the systemic form. The course of urticaria pigmentosa depends on the age of the patient. The spots in children usually disappear again at the age of two to three years. A chronic form occurs only rarely. This can cause problems even for adults. But even in adults, the prognosis is usually positive.
The exact complications and symptoms of urticaria pigmentosa depend very much on the severity of the disease, so that a general prediction is usually not possible. However, patients suffer from spots that appear on the skin. These spots are reddish or brownish and have a negative effect on the aesthetics of the person concerned.
For this reason, many suffer from inferiority complexes or are ashamed of the disease. Children can be bullied or teased for this reason. There is also severe itching. Urticaria pigmentosa can also lead to severe headaches or diarrhea and vomiting. In severe cases, this leads to unconsciousness, which can injure the person concerned.
The disease can also lead to breathing difficulties, so that the patient’s quality of life is significantly restricted and reduced. Treatment of urticaria pigmentosa is carried out with the help of drugs. Since it cannot be carried out causally, those affected are dependent on taking the medication. Usually there are no complications. The patient’s life expectancy is also not reduced by the disease.
When should you go to the doctor?
Urticaria pigmentosa should always be treated medically by a doctor. Further complications can only be prevented by proper treatment, as it cannot heal on its own. Early diagnosis always has a very positive effect on the course of the disease and can also prevent further deterioration of the symptoms. A doctor should be contacted if the affected child suffers from severe headaches, vomiting or diarrhea over a longer period of time.
Severe breathing difficulties can also indicate the disease, with some patients even losing consciousness. If these symptoms occur for no particular reason and do not go away on their own, you must see a doctor. The disease can be diagnosed and treated by a pediatrician or general practitioner.
There are no particular complications, so that the life expectancy of the child is usually not reduced by urticaria pigmentosa. If urticaria pigmentosa is a chronic disease, regular visits to a doctor are advisable to monitor the condition of the internal organs.
Treatment & Therapy
Urticaria pigmentosa does not always have to be specially treated because it often goes away on its own in children. In adult patients, it is important to find out the exact cause. The triggers that cause the mast cells to be released must be consistently avoided. These usually include friction, stress, abrupt temperature changes or insect bites.
Complete healing of mastocytosis is not possible. However, the symptoms of the disease can be effectively relieved by administering drugs such as antihistamines. These inhibit the effect of the released histamines. Furthermore, so-called mast cell stabilizers can be used, which ensure that the mast cells release less histamine. For this purpose, the patient usually receives metered dose aerosols, nose drops or eye drops.
Urticaria pigmentosa cannot be prevented directly. However, there is the possibility of avoiding the triggering stimuli, for example by the patient not taking special medication or food.
The scope of aftercare measures for urticaria pigmentosa (UP) is determined by the respective clinical picture and age of the person affected. In affected children, for example, urticaria pigmentosa in early childhood (up to three years) can lead to spontaneous blistering on the skin. Due to external stimuli (e.g. heat/cold or infections), the blisters burst regularly. The task of aftercare is then to prevent scarring by applying healing ointments.
Basically, urticaria pigmentosa cannot be cured. In the further development of the affected children, the mast cells in the body do not multiply properly. There are too many. In 95 percent of all cases of the disease, too many mast cells manifest themselves as reddish-brown to brown spots on the affected person’s skin. When exposed to external stimuli, the spots release histamine. Aftercare then focuses on treating the symptoms triggered by the histamine.
Depending on the severity of the symptoms (e.g. seizure-like drop in blood pressure or anaphylaxis), hospital stays may also be necessary to regenerate the state of health. The affected person himself can relieve symptomatic external skin irritations (e.g. itching, swelling or redness) by applying cortisone ointments and treat sudden headaches or body aches with medication. In the remaining five percent of all cases of illness, those affected have no symptoms. There is then no need for follow-up measures.
You can do that yourself
Urticaria pigmentosa requires medical clarification. The skin condition can be treated with various home remedies and self-help measures.
The first thing to do is to cool the affected regions sufficiently. Abrupt temperature changes and mechanical irritation should be avoided. The medication prescribed by the doctor must be taken according to the doctor’s instructions. At the same time, possible triggers must be eliminated. Causative drugs such as codeine, procaine, or polymyxin B must be checked and discontinued if necessary. In addition, a comprehensive physical examination is necessary, as there may be undetected concomitant diseases.
Itching or redness can also be treated with various natural remedies. Graphites D12 can be used for dry skin. Severely itchy rash is treated with Petroleum rectficatum D12. The use of alternative means should first be discussed with the responsible doctor.
In the case of chronic urticaria pigmentosa, lifestyle habits must be adjusted. Diet, personal hygiene and physical activity influence the course of the disease. A healthy and balanced diet without irritating substances applies. Patients should care for their skin daily and take it easy otherwise. The responsible dermatologist explains which self-help measures make sense in detail.