Urticaria Pigmentosa

Urticaria Pigmentosa is a rare skin condition that occurs mostly in kids. It is the most common form of Mastocytosis or mastocytoma. Although around 75% of cases occur amongst infants and children but it can also occur in adults.

What is Urticaria Pigmentosa?

Mast cells are part of the body’s immune system. They contain histamine which triggers inflammatory reactions when germs invade the body.

In this condition, there is an accumulation of mast cells in the skin. When the skin is rubbed, the histamine gets triggered and releases into the skin. This causes the nearby tissues to get swollen and infected and produces hive like lesions called Darier’s Sign.

Causes of Urticaria Pigmentosa

The exact cause of Urticaria pigmentosa is not known as this is such a rare condition. It could be genetic where the child inherits an abnormal gene or undergoes a gene mutation. But research is not conclusive on the genetic link in causing this condition.

However, there are certain environmental conditions that could trigger the condition. They are:

  • Rubbing the skin
  • Infections
  • Physical exercise
  • Emotional and mental stress
  • Hot liquids
  • Spicy foods like pepper and chilli
  • Extreme weather conditions like harsh sunlight or very cold weather
  • Certain drugs that contain codeine, morphine or aspirin
  • Venom produced by bee stings
  • Alcohol

Symptoms of Urticaria Pigmentosa

Urticaria Pigmentosa is characterized by the presence of:

  • Dark patches on skin
  • Itchy sensations of different intensities
  • When the skin is rubbed, it breaks out into lesions which seem to resemble hives. These are called Darier’s Sign.
  • Flushing or skin redness
  • It can be accompanied by other symptoms such as diarrhea, headache, rapid heartbeat, nausea, dizziness and wheezing.
  • The spread can occur all over but is usually found on the trunk the most, although it appears on the face and limbs too.

Prognosis of Urticaria Pigmentosa

This condition is very rare and is found more in infants and children. However, there are no complications if this develops during childhood and often goes away by the time puberty is reached.

A very small percentage does not outgrow the symptoms and the lesions remain even as they grow into adulthood.

However, adults can have complications and it could result in a systemic form of Urticaria Pigmentosa which can get serious.

Complications of Urticaria Pigmentosa in adults

In adults, the accumulation of mast cells can lead to systemic mastocytosis where the mast cells start accumulating in other organs. This then spreads in these organs like the spleen, liver, bone marrow and lymph nodes even. This is however a rare condition.

In extremely rare cases, the spread could turn cancerous. It could lead to the development of mast cell leukemia or mast cell sarcoma. This is where the check up and proper diagnosis by the doctor becomes important.

Diagnosis of Urticaria Pigmentosa

The doctor will check and examine the lesions to diagnose the condition correctly. The presence of the Darier’s Sign will be checked to confirm the presence of Urticaria Pigmentosa.

Some other skin conditions like melanoma and basal cell carcinoma can be mistaken for this. Therefore, the doctor may ask for a skin biopsy to be undertaken in order to rule out cancer. A small portion of the skin will be scraped off and sent to the diagnostic lab for testing to check if it is cancerous or not.

Apart from this, blood tests and urine histamine tests may also be asked to be carried out.

Treatment of Urticaria Pigmentosa

As the exact cause of Urticaria Pigmentosa is unknown, complete treatment cannot be done. Most of the treatment procedures aim at relieving symptoms but the chance of relapse is very high. The doctor will recommend the treatment according to the severity and number of lesions.

Some of the more common treatments are:

  1. Antihistamines – these are taken to relieve the itching and flushing of the skin.
  2. Topical corticosteroids – They are either in a gel form or cream form and are applied to the affected areas. They contain anti-inflammatory properties and are mainly to soothe the skin.
  3. Oral disodium cromoglycate – This is for the more severe symptoms. This medication not only relieves the itching and flushing, but also improves the other systemis symptoms such as diarrhea and stomach cramps.
  4. Photochemotherapy – This is a form of light therapy which contains UV radiation. It is used to reduce the number of mast cells. This has proven to be more effective than the above two methods but relapse can still happen.
  5. Intralesional injections – This is recommended in serious cases but it comes with a risk of skin atrophy. Therefore, it cannot be performed for a long duration.

Side effects of treatment

The treatment for Urticaria Pigmentosa can have some side effects, especially if corticosteroids are being used. It could cause insulin resistance where the body starts growing used to insulin and begins to resist it. It could also lead to glucose intolerance and red skin syndrome because of the flushing of the skin.

Prevention of Urticaria Pigmentosa

As this form of skin disorder is very rare and the exact cause is unknown, there is no sure way to prevent it. However, certain precautions can be taken to prevent it from aggravating. They are:

  • Try avoiding the urge to itch or scratch the skin. If this is a child, it could be a little tricky. Gloves can be worn so that the skin does not get torn with excessive scratching. It is better to wear these gloves to bed as well.
  • Nails must always be cut to avoid spread of infections
  • Avoid hot baths and showers as they dry out the skin and aggravate itching.
  • Wear light, cotton clothing
  • Avoid alcohol or any medications that could make this condition flare up
  • Avoid hot liquids and spicy foods.

Urticaria Pigmentosa Pictures

Image of Urticaria Pigmentosa Photo of Urticaria Pigmentosa

Picture of Urticaria Pigmentosa Urticaria Pigmentosa Picture

Urticaria Pigmentosa is a rare skin condition where lesions appear. It is characterized by an itching sensation. The exact cause is unknown but many environmental factors like stress or hot liquids can aggravate it. Treatments mainly involve symptom relief. This condition is more common among children.

Medical References:

healthline.com/health/urticaria-pigmentosa

medlineplus.gov/ency/article/001466.htm

nytimes.com/health/guides/disease/urticaris-pigmentosa/overview.html

clinuvel.com/science-of-skin/skin-conditions/rare-skin-conditions/urticaria-pigmentosa-up

skinsupport.org.uk/conditions-details/urticaria-pigmentosa

aboutkidshealth.ca/En/HealthAZ/ConditionsandDiseases/Dermatology/Pages/urticaria-pigmentosa.aspx

derm101.com/therapeutic/urticaria-pigmentosa-mastocytosis/

mddk.com/urticaria-pigmentosa.html

scripps.org/artcles/2202-urticaria-pigmentosa

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