Urticaria Pigmentosa – What is it, Symptoms and Treatments!

Urticaria Pigmentosa – What it is, Symptoms and Treatments of this condition. Furthermore, Urticaria Pigmentosa is 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. Its job is to produce inflammation , releasing a substance called histamine in response to germs and other invaders. In Urticaria Pigmentosa , there are many masts on your skin .

This disease is most commonly seen in babies and children, but adults can be affected as well. The main symptom is the dark colored lesion on the skin . The lesions can be very itchy and difficult not to scratch.

When rubbed or scratched, the lesions respond with a Darier sign. Darier’s sign looks similar to beehives. It is caused by the release of histamine from mast cells.

In most children, Urticaria Pigmentosa departs from puberty. Usually, complications are seen in older children or adults. Rarely, Urticaria Pigmentosa can progress to systemic mastocytosis in an adult.

In systemic mastocytosis, mast cells can 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 .

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

Symptoms of Urticaria Pigmentosa can include:

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

Adults or teenagers 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 of Urticaria Pigmentosa is 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 of Urticaria Pigmentosa is very rare, with only about 50 documented cases.

Doctors know that when 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. In Urticaria Pigmentosa , there is no invader. The immune response results in itchy skin lesions .

Diagnoses of Urticaria Pigmentosa: The diagnosis of Urticaria Pigmentosa is based on observation of the lesions . Darier’s sign is a classic symptom implicating Urticaria Pigmentosa and most lesions are similar in color. Lesions that appear different from others can be a sign of cancer .

• Possible cancers can include:
• Melanoma (the deadliest of skin cancer )
• Basal cell carcinoma (uncontrolled growth or damage to the outer layer of the skin )
• Actinic keratosis (a scaly, scaly bump of skin caused by years of sun exposure)

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

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

Treatment options include:

• Antihistamines to relieve itching and redness of the skin
• Topical corticosteroids (gel or cream with anti-inflammatory properties)
• Intralesional corticosteroids (injection with anti-inflammatory steroid drugs)
• Hydrocolloid dressings (acts as a cure to keep the medication on the skin )
• Fluocinolone Net Acetate (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
• Don’t touch the bubbles (no matter how tempting)
• Don’t scratch the lesions (this will only send out more histamines creating a bigger reaction)

People with Urticaria Pigmentosa should avoid certain medications, including:

• Aspirin
• Codeine
• Opiates (morphine and codeine)

Alcohol intake should be limited or eliminated completely as it can be a trigger for Urticaria Pigmentosa .

Complications of Urticaria Pigmentosa: Most cases of Urticaria Pigmentosa affect only the skin . Cases where Urticaria Pigmentosa affects other organs are usually found in older children and adults.

Organs that can be affected by Urticaria Pigmentosa include:

• Liver
• Spleen
• bone marrow

Unfortunately, treatment for Urticaria Pigmentosa can have some unwanted side effects. Side effects of prolonged treatment include:

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

Urticaria Pigmentosa Perspective: Most cases of Urticaria Pigmentosa appear in children. As they get older, most will outgrow the disease. Lesions usually disappear as a child moves into adulthood. Up to 25% do not overcome the disease and maintain the lesions into adulthood.

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

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

• Avoid hot baths to avoid drying out your skin and making itching worse
• Avoid itchy, irritating clothes – try cotton or other lightweight fabrics instead
• Keep your nails short
• wear light cotton gloves to sleep to avoid scratching

Your pediatrician may have more tips. Most cases of Urticaria Pigmentosa  disappear by the time the child becomes an adolescent.