Urticaria pigmentosa is the name given to a form of mastocytosis. It is predominantly seen in young children.
What is urticaria pigmentosa?
Urticaria pigmentosa is the most common form of mastocytosis. Mastocytosis refers to a rare disorder in which mast cells accumulate in the skin as well as in internal organs. In medicine, urticaria pigmentosa, which is a benign disease, is also called cutaneous mastocytosis. It differs from systemic mastocytosis in that the mast cells accumulate exclusively in the skin. In the systemic form, however, organs such as the liver, spleen and intestines are also affected. In addition, the systemic form predominantly affects adults, whereas urticaria pigmentosa is primarily seen in children. The disease is not contagious. Mast cells are white blood cells. They are found in every human being and contain substances such as heparin, serotonin, histamine as well as various enzymes that are among the mediators of inflammation. The mast cells perform the function of triggering 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, widening of the skin vessels, redness and itching. Furthermore, wheals develop. For the first time, urticaria pigmentosa usually appears during the first 24 months of life.
Causes
Urticaria pigmentosa or cutaneous mastocytosis results from the accumulation of mast cells in the skin. However, to date it has not been possible to clarify what causes this accumulation. Some physicians suspect the reason for the disease to be a genetic defect that triggers uncontrolled growth of the mast cells. There is talk of 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 in many affected children no changes in the c-KIT gene could be detected. The triggers for the release of mast cell contents vary from patient to patient and depend on the form of mastocytosis. In urticaria pigmentosa, for example, these can be cold, heat, friction, infections or stress. Likewise, food additives, hot spices, allergies, insect venoms, alcohol, and the use of certain medications such as acetylsalicylic acid, quinine, codeine, or narcotics are all possible causes. Basically, urticaria pigmentosa is a pseudoallergy from which the symptoms are triggered.
Symptoms, complaints, and signs
Symptoms vary greatly from individual to individual in urticaria pigmentosa. Thus, they can be both minor and severe. In most cases, the disease affects children, in whom reddish-brown spots appear on the skin, usually either on the trunk or on the thighs. Furthermore, there is redness and swelling on the skin and blisters. When rubbing the patches, itching often becomes noticeable. Later, wheals appear. In severe cases of skin mastocytosis, headache, diarrhea, vomiting and breathing difficulties occur. Even unconsciousness is possible. However, systemic mastocytosis, which also affects the internal organs, occurs in only about 10 percent of all patients.
Diagnosis and course of the disease
If urticaria pigmentosa is suspected, the physician first conducts an interview with the patient. In addition, he is guided by the typical mastocytosis symptoms such as mottling, wheals and itching. In order to make a reliable diagnosis, the physician performs a Darier test. This involves scratching or rubbing over an affected area of skin. If the area reddens or itches after a few seconds, the Darier test is considered positive and the suspicion of cutaneous mastocytosis has been confirmed. Furthermore, it is possible to take a skin sample, the examination of which takes place in a pathological laboratory. Likewise, a blood test can be performed to determine the triptase value. In the case of cutaneous mastocytosis, the triptase value is usually normal, whereas it is higher in the systemic form. The course of urticaria pigmentosa depends on the age of the patient.In children, the spots usually disappear at the age of two to three years. A chronic form rarely occurs. This can still cause problems even in adults. But even in adults, the prognosis is usually positive.
Complications
The exact complications and symptoms in urticaria pigmentosa depend very much on the severity of the disease, so 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 affected person. For this reason, many affected individuals suffer from inferiority complexes or are ashamed of the condition. In children, bullying or teasing may occur for this reason. Furthermore, severe itching also occurs. Urticaria pigmentosa can also lead to severe headaches or to diarrhea and vomiting. In severe cases, unconsciousness may occur, during which the affected person may injure himself. The disease can also lead to breathing difficulties, so that the patient’s quality of life is significantly restricted and reduced. Urticaria pigmentosa is treated with the help of medication. Since it cannot be carried out causally, those affected are dependent on taking the medication. As a rule, there are no complications. The life expectancy of the patient is also not reduced by the disease.
When should you see a doctor?
Urticaria pigmentosa should always be treated medically by a doctor. Proper treatment is the only way to prevent further complications, 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 worsening of the symptoms. A doctor should be contacted if the affected child suffers from severe headaches, vomiting or diarrhea over a long period of time. In this case, severe breathing difficulties may also indicate the disease, and some patients may even lose consciousness. If these symptoms occur without any particular reason and do not disappear on their own, a doctor must be consulted in any case. The disease can be detected and treated by a pediatrician or by a general practitioner. Special complications do not occur, so that as a rule the life expectancy of the child is not reduced by Urticaria pigmentosa. If urticaria pigmentosa is a chronic disease, regular visits to a physician are advisable to monitor the condition of the internal organs.
Treatment and therapy
Urticaria pigmentosa does not always require special treatment because it often resolves on its own in children. In adult patients, it is important to determine the exact cause. For example, the triggers for the release of mast cells must be consistently avoided. These usually include friction, stress, abrupt temperature changes or insect bites. A complete cure of mastocytosis is not possible. However, the symptoms of the disease can be effectively alleviated by administering drugs such as antihistamines. These inhibit the effect of the histamines released. In addition, so-called mast cell stabilizers can be used to ensure that the mast cells release fewer histamines. For this purpose, the patient usually receives metered-dose inhalers, nasal drops, or eye drops.
Prevention
Urticaria pigmentosa cannot be prevented directly. However, it is possible to avoid the triggering stimuli, for example, by having the patient avoid specific medications or foods.
Aftercare
The extent of follow-up care for Urticaria Pigmentosa (UP) is determined by the individual’s clinical picture and age. In affected children, for example, urticaria pigmentosa may cause spontaneous blistering of the skin in early childhood (up to three years of age). Due to external stimuli (for example heat/cold or infections) the blisters burst regularly. The task of aftercare is then to prevent scarring by applying healing ointments. Urticaria Pigmentosa is basically not curable. In the further development of the affected children, the mast cells in the body do not multiply properly. There are too many.The excessive number of mast cells manifests itself in 95 percent of all cases of the disease as reddish-brown to brown spots on the skin of the affected person. In response to external stimuli, the patches release histamine. Follow-up care then focuses on treating the symptoms triggered by the histamine. Depending on the severity of the symptoms (for example, seizure-like drop in blood pressure or anaphylaxis), clinical stays may also be necessary to regenerate the health condition. The affected person can relieve symptomatic external skin irritations (for example itching, swelling or redness) by applying cortisone ointments and treat sudden headaches or pain in the limbs with medication. In the remaining five percent of all cases, those affected have no complaints. There is then no need for follow-up measures.
What you can do yourself
Urticaria pigmentosa requires medical clarification. The skin condition can be treated with various home remedies and self-help measures. First, it is important to cool the affected regions sufficiently. Abrupt temperature changes and mechanical irritation should be avoided. Medically prescribed medication must be taken according to the physician’s instructions. Accompanying this, possible triggers must be eliminated. Causative medications such as codeine, procaine or polymyxin B must be checked and discontinued if necessary. In addition, a comprehensive physical examination is necessary, since there may be undetected concomitant diseases. Treatment of itching or redness is also done using various natural remedies. Graphites D12 can be used for dry skin. Strongly itchy rash is treated by means of Petroleum rectficatum D12. The use of alternative remedies should first be discussed with the responsible physician. 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 should be followed. Patients should care for their skin daily and otherwise take it easy. The dermatologist in charge will explain which self-help measures are useful in detail.
