Diseases And Treatments

Urticaria Pigmentosa - What It Is, Symptoms and Treatments


Urticaria Pigmentosa - What It Is, Symptoms and Treatmentsthis condition. In additionUrticaria Pigmentosais an allergy-mediated skin condition that causes discolored lesions and itchy skin. The condition is characterized by the presence of many mast cells in the skin. Mast cells are part of your immune system. Their job is to produce inflammation by releasing a substance called histamine in response to germs and other invaders. InUrticaria Pigmentosa, there are many masts in your skin.

This disease is most commonly seen in infants and children, but adults may also be affected. The main symptom is the dark-colored lesion on the skin. Lesions can be very pruritic and difficult to scratch. When rubbed or scratched, the lesions respond with a sign of Darier. Darier's sign looks similar to hives. It is caused by the release of histamine from mast cells.

In most children,Urticaria Pigmentosamoves away from puberty. Usually complications are seen in older children or in adults. Rarely, theUrticaria Pigmentosa

can progress to systemic mastocytosis in an adult. In systemic mastocytosis, mast cells may accumulate in other organs of the body. In extremely rare cases, this can result in mast cell leukemia or mast cell sarcomas, which are both forms of cancer.



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Symptoms of Urticaria Pigmentosa:The main symptom ofUrticaria Pigmentosaare brownish lesions on the skin. Rubbing the lesions releases histamines that produce intense itching along with blisters or hives (Darier's sign).

Symptoms ofUrticaria Pigmentosamay include:

  • Pruritus (itching that varies in severity and intensity)
  • Flushing (redness of the skin)
  • On lesion pigmentation (very dark coloration of lesions)

Adults or teens are more likely to have unusual symptoms. These include:

  • Diarrhea
  • Tachycardia (rapid heart rate)
  • Nausea or vomiting
  • Faint
  • Dizziness
  • Headache

Causes of Urticaria Pigmentosa:The exact cause ofUrticaria Pigmentosais unknown. There may be a genetic cause for some cases (the child inherits an abnormal gene from one of their parents, or there is a genetic mutation), while other cases appear for no reason. The hereditary form ofUrticaria Pigmentosais very rare, with only about 50 documented cases.

Doctors know that when the lesions are rubbed, they release histamines. Histamines are chemicals that initiate an immune response. Normally, the immune system is activated in response to germs or other invaders. AtUrticaria Pigmentosa, there is no intruder. The immune response results in pruritic skin lesions.


Diagnosis of Urticaria Pigmentosa:The diagnosis ofUrticaria Pigmentosais based on the observation of the lesions. The Darier's sign is a classic symptom that implies theUrticaria Pigmentosaand most of the lesions have similar colors. The lesions that appear different from the others may be a sign of cancer.

  • Possible cancers may include:
  • Melanoma (the most deadly skin cancer)
  • Basal cell carcinoma (uncontrolled growth or lesions in the outer layer of the skin)
  • Actinic keratosis (a presumptive, flaky birth of skin caused by years of sun exposure)

Your doctor will test any unusual cancer lesions. This will require a small skin sample for microscopic examination and testing. A skin biopsy will be recommended for this purpose.

Treatments of Urticaria Pigmentosa:There are no cures forUrticaria Pigmentosa. Treatment focuses on easing symptoms and controlling injuries. Your doctor will recommend a specific treatment based on the number of lesions and their tolerance. For example, painless and easy-to-apply treatments may be best for young children.


Treatment options include:

  • Antihistamines to relieve itching and flushing of the skin
  • Topical corticosteroids (gel or cream with anti-inflammatory properties)
  • Intra-lesional corticosteroids (injection with anti-inflammatory steroid drugs)
  • Hydrocolloid dressings (act as a cure to keep the medication on the skin)
  • Fluocinolone acetoid (a synthetic corticosteroid)
  • Chlorpheniramine maleate (antihistamine used to control allergic reactions)

In adults, a form of light therapy called photochemotherapy with ultraviolet radiation has proven to be an effective treatment.

In order to encourage recovery:

  • Do not rub the skin
  • Do not touch the bubbles (no matter how tempting)
  • Do not scratch the lesions (this will only send more histamines creating a bigger reaction)

People withUrticaria Pigmentosashould avoid certain medications, including:

  • Aspirin
  • Codeine
  • Opiates (morphine and codeine)

Alcohol intake should be limited or eliminated completely, as it can be a trigger forUrticaria Pigmentosa.



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Complications of Urticaria Pigmentosa:Most of the casesUrticaria Pigmentosaaffects only the skin. Cases whereUrticaria Pigmentosaaffects other organs usually found in older children and adults.


The organs that may be affected byUrticaria Pigmentosainclude:

  • Liver
  • Spleen
  • Bone marrow

Unfortunately, treatment forUrticaria Pigmentosamay have some unwanted side effects. Side effects of long-term treatment include:

  • Red skin syndrome (corticosteroid withdrawal)
  • Diabetes mellitus (glucose intolerance due to chronic use of steroid therapy)
  • Resistance to insulin (the body grows immune to the presence of insulin)

Perspective of Urticaria Pigmentosa:Most of the casesUrticaria Pigmentosaappears in children. As they get older, most will outgrow the disease. The lesions usually disappear when a child moves into adulthood. Up to 25% do not exceed the disease and keep the lesions into adulthood.


Prevention of Urticaria Pigmentosa:There is no safe way to preventUrticaria Pigmentosa. The hereditary form is very rare, and even when the child has the abnormal gene, they may not developUrticaria Pigmentosa.

However, you can prevent the disorder from getting worse. Try to help your child not scratch or rub irritated skin to prevent the lesions from spreading:

  • Avoid hot baths to avoid drying the skin and worsening the itching.
  • Avoid itching, irritating clothes - try cotton or other lightweight fabrics instead
  • Keep your nails short
  • wear lightweight sleeping cotton gloves to avoid scratching


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Your pediatrician may have more tips. Most of the casesUrticaria Pigmentosadisappear until the child becomes a teenager.