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Urticaria Pigmentosa: What is it, Symptoms, and Treatment

What is Urticaria Pigmentosa?

Urticaria pigmentosa is a type of mastocytosis, that belongs to a rare group of diseases. They are caused by the assembling of a large number of mast cells on the skin surface. These mast cells release histamine (a variety of biologically active) substances. This disease was first studied by scientists in 1986.

In the literature, family cases of the evaluation of this disease are well described. But at present, its genetic inheritance has not been proven. This disease occurs equally in both males and females. Quite often occurs in childhood in the form of a skin rash.

This disease usually begins to occur during the first years of a child’s life. In most cases spontaneously regresses during teen age. At the beginning of the development of the disease, dirty-red or red-brown spots begin to appear on the skin. 

These spots later turn into blisters and can merge with each other. When the blisters end, scarring forms in this place in the form of pale pink scar tissue.

Blisters form on the baby’s skin with severe itching. The spots have a round shape with a clear shape, up to 8 mm in diameter.

Mastocytosis

Most experts think that this disease is associated with an injury to the reticulohistiocytosis system. At the moment, the role of heparin and histamine is well studied. It can occur by the process of degranulation in mast cells under the impact of immune and non-immune activators.

This disease in 70% of cases develops before the age of 2-2.5 years. In young children, the skin is mostly affected. A complete revert of this disease is possible during the puberty period in most cases.

Mastocytosis in adults, often occurs as vast symptoms with damage to internal organs.

There are several forms of mastocytosis:

  1. Urticaria pigmentosa
  2. nodular mastocytosis
  3. Mastocytoma
  4. diffuse mastocytosis
  5. Erythrodermic type
  6. Spotted telangiectasia
  7. bullous form
  8. atrophic form

Clinical symptoms occur in the following percentages:

  • 75-80% damage to the skin
  • 5-7% skeletal system
  • 10% – systemic mastocytosis
  • 2% mast cell sarcoma
  • 1% aggressive form

In this disease symptoms of rashes occur on open areas of the body. However, there are some cases in addition to skin lesions. For example, a drop in blood pressure and obstruction in the bronchi.

These conditions result in difficulty in breathing. Such victims often lose consciousness and need emergency medical attention.

Urticaria Pigmentosa Symptoms

An acute form of the disease can occur with the sudden appearance of a severe itchy rash on the body. A painful condition can occur by diarrhea and vomiting, and high body temperature. 

Blisters can occur at any part of the body such as lips, tongue, nasopharynx, or larynx. These rashes can cause the patient to have difficulty breathing and eating. The acute form of the disease is symptoms of an allergic reaction to certain drugs or food.

A very dangerous disease occurs in the form of acute Quincke’s edema. Quincke’s edema can last from several hours to several days. Edema occurs of soreness and the severity of pain.

If the swelling extends to the mucous areas of the internal organs and skin. Then this sometimes leads to disorders of the digestive system, vision, and respiration. In severe cases, death from asphyxia of the patient is possible. 

Other Symptoms

In addition to Darier signs you may also experience the following symptoms:

  • Racing heartbeat
  • Scalp Face
  • Diarrhea
  • Flushing (redness of the skin)
  • Wheezing
  • Fainting
  • Headache
  • Drop in blood pressure
  • Difficulty breathing
  • Swelling of the throat and oral cavity
  • Severe pain in the abdomen
  • Loss of consciousness

The spots occur on the skin, but in some places, they can unite. Spots on the skin can combine with the formation of nodules. Most of the rashes can locate on the skin of the trunk, scalp, skin of the hands, and feet.

When a child or adult has damage to internal organs. There may be a violation of the function of the affected organ. When the skeletal system is affected, severe pain develops in the joints and bones. 

In the initial stages of damage to the liver and spleen, there are no clinical symptoms. But in the future, hepatitis develops with symptoms of portal hypertension and ascites.

Urticaria pigmentosa in adults indicates an increase in the severity of the disease. First, spots and papules appear on the chest and then spread to the skin of the arms, legs, and face. Damage to internal organs for several years without proper treatment can cause death. Therefore, as soon as you find urticaria pigmentosa symptoms, immediately consult a doctor.

Triggers

You should choose foods and drinks carefully and avoid anything that triggers reaction. If ultraviolet rays are a trigger factor in allergies, then this is solar urticaria. A painful condition feels during a period of high practice of ultraviolet rays. More often people suffer from impaired porphyrin metabolism and a weakened liver.

People with excessive sensitivity to ultraviolet radiation should avoid direct sunlight. The other some triggers include:

  • Alcohol
  • Aspirin
  • Thiamine 
  • Quinine
  • Opiates
  • Dextromethorphan
  • Anesthesia medicines
  • X-ray dyes

Some other are:

  • Rubbing the skin with towels
  • Exercise
  • Cold exposure
  • Sunlight
  • Spicy food
  • Hot baths
  • Hot drinks

If ultraviolet rays are a trigger factor in allergies, then this is solar urticaria. A painful condition feels during a period of high practice of ultraviolet rays. More often people suffer from impaired porphyrin metabolism and a weakened liver.

People with excessive sensitivity to ultraviolet radiation should avoid direct sunlight. They should choose foods and drinks carefully that do not provoke a reaction.

Diagnosis

Diagnosis of the disease is based on the test and the collection of the patient medical history. The allergist will perform the test with a strong rubbing of any focus skin patches. As a result, erythema and swelling of the rash will appear.

He may perform a general blood test with a count of the number of mast cells in the blood. An ultrasound examination of the internal organs, and the human lymphatic system. 

Additionally, he can perform an X-ray examination of the bone apparatus. Of great diagnostic importance is the histological examination of the material. 

To determine mast cells, the immunohistochemical reaction method is also used. In which there is the use of antibodies against specific mast cell antigens.

Treatment of Urticaria Pigmentosa

Treatment of this disease depends on the removal of symptoms that cause a problem for the patient. If y children develop urticaria pigmentosa during the first two or three years of life. A complete recovery observes in 80% of cases with properly selected therapy. Recovery usually occurs by the time the child reaches a teen.

If the disease begins to manifest itself during the teen of a child. Then a cure observes in 56% of cases, and 44% may develop a systemic form of the disease with damage to internal organs.

Urticaria pigmentosa doesn’t need treatment until there is no systemic involvement. In some case, the allergist prescribe the following treatment.

  • Antihistamine
  • Anti-serotonin
  • Corticosteroid creams
  •  EpiPen

You can also treat the disease with the help of immunostimulating drugs. Which, according to their mechanism of action, can increase the body’s immune response. 

Complication

Another type of disease is persistent Papular urticaria. It occurs by the allergic reaction to insect bites like mosquitoes and other insects. In this condition, rashes convert into papules, puffiness, acanthosis, and hyperkeratosis. A nodular red-brown rash affects the elbow and knee bends of patients.

It can last up to several months, and even with a small impact of allergens on the body, flare up again and again. In this case, the rash spreads to any part of the skin, although these irritations are mild than in the acute form.

Constant itching leads to insomnia and nervous disorders. Often, patients suffer from headaches or joint pain, diarrhea, and vomiting. The causes of chronic disease are often an infectious system that takes a long time in the patient’s body. Therefore, to prevent the disease, treat the infected parts in the internal organs.