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Maculopapular cutaneous mastocytosis

What is it maculopapular cutaneous mastocytosis?

Maculopapular cutaneous mastocytosis is also called urticaria Pigmentosa is the most common type of cutaneous mastocytosis, a condition in which there are brown spots or freckles on the skin due to abnormal collections of mast cells.

Maculopapular cutaneous mastocytosis in children.

Maculopapular cutaneous mastocytosis most often affects babies, with the first patches appearing at a few months of age. The patches are often mistaken for moles or insect bites at first, but the lesions persist and gradually increase in number over several months or years. They can appear anywhere on the body, including the scalp, face, trunk, and extremities.

In young children, it is common for mastocytosis patches to blister when rubbed. If many patches are rubbed or spontaneously activated at the same time, the baby may become irritated, but serious symptoms rarely occur.

Over time, mastocytosis becomes less itchy and eventually the patches fade. By adolescence, most of the patches will be gone.

Maculopapular cutaneous mastocytosis in children.

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Urticaria pigmentosa

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Diffuse cutaneous mastocytosis

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Diffuse cutaneous mastocytosis

Maculopapular cutaneous mastocytosis in adults.

Maculopapular cutaneous mastocytosis can sometimes develop for the first time in an adult. Only a few injuries may appear, or many. Lesions can be unsightly and itchy. Unfortunately, in adults, urticaria pigmentosa tends to persist over the long term. Sometimes patients can have systemic symptoms (such as redness, itching, low blood pressure, anaphylactic shock, diarrhea, and bleeding from the gastrointestinal tract), suggesting the presence of systemic mastocytosis.

Located Rarely, has cutaneous mastocytosis been reported to arise in a radiotherapy area for the breast Cancer for unknown reasons

Maculopapular cutaneous mastocytosis in adults.

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Maculopapular cutaneous mastocytosis. Urticaria pigmentosa

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Maculopapular cutaneous mastocytosis. Urticaria pigmentosa

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Diffuse cutaneous mastocytosis

What precautions should I take?

Exercise or heat can aggravate the symptoms of cutaneous mastocytosis. A severe reaction can cause redness and fainting.

Certain medications can cause mast cells degranulation and should be avoided if there is extensive urticaria pigmentosa. These include:

  • Aspirin (salicylates) and other non-steroidal anti-steroidsinflammatory drugs
  • Codeine and morphine (narcotics)
  • Alcohol
  • Anticholinergics

People with urticaria pigmentosa can have severe allergic reaction from bee or wasp stings, therefore they may need to carry an injectable epinephrine / adrenaline kit.

What research should be done?

The appearance of maculopapular cutaneous mastocytosis is generally so characteristic that specific tests are not necessary. However, occasionally a skin biopsy it is necessary to confirm the diagnosis (see maculopapular cutaneous mastocytosis) pathology) If there are any symptoms suggesting internal involvement (systemic mastocytosis), see our page on mastocytosis for more information. Tests may include:

  • Blood cell count
  • Serum tryptase
  • DEXA bone scan
  • Bone marrow examination

What is the treatment for maculopapular cutaneous mastocytosis?

Maculopapular cutaneous mastocytosis is not serious, and does not require any treatment in most cases (unless there is also systemic compromise). The following may be useful.

  • Oral antihistamines
  • Current steroids
    Powerful steroid creams applied for several months under occlusion It can reduce itching and unsightly, but the patches tend to reappear within a few months. Topical steroids are only suitable for limited areas.
  • Topical calcineurin inhibitors, such as pimecrolimus. cream
  • Phototherapy
    This way of Ultraviolet radiation It is the most effective treatment for adults with maculopapular cutaneous mastocytosis. Two or three treatments per week for several months are required. Narrowband UVB or (if available) photochemotherapy (PUVA) reduces itching and improves appearance. Cutaneous mastocytosis is likely to recur within six to twelve months.