Urticaria Pigmentosa

Skin manifestations:

• Red-brown bumps ranging from 1 to 5 cm (mastocytoma)
• Red-brown spots or bumps on the skin (mastocytosis)
• Bumps that swell, turn red, and itch when rubbed
• Blisters on the skin
• Hives (urticaria)
• Persistent brown discoloration after redness fades

General complaints:

• Hot flashes
• Dizziness
• Nausea and vomiting
• Headaches
• Palpitations
• Tingling
• Diarrhea
• Fainting

Mastocytosis of the skin is classified into several groups:

1. Diffuse cutaneous mastocytosis

• Redness and thickening of the entire skin
• Often bumps at the site of redness
• Blister formation with minimal friction, which can occur after a viral infection or teething
• Often no spread to organs
• Can be hereditary

2. Maculopapular cutaneous mastocytosis (urticaria pigmentosa)

• Polymorphic (in children):

• Large red-brown oval spots, bumps, blisters of varying sizes. These skin lesions may continue to grow until the age of 10 and often disappear spontaneously around age 16.
• Darier’s sign: Rubbing the affected skin causes itching and swelling. Skin lesions may form blisters from friction until the age of 3.

• Monomorphic (adults and children > 3 years):

• Multiple oval brown-red skin lesions that all look the same. In this form, there may also be an accumulation of mast cells in other organs (systemic mastocytosis). The exact percentage of people with mast cells in their organs is unknown.
• Starts on the hips, armpits, or lower back, and years later spreads to the rest of the body. Does not appear on the face.
• Often between the ages of 20 and 35
• Often also mastocytosis in the organs, although this rarely causes complaints

Systemic mastocytosis is an accumulation of mast cells in the skin and organs. This form is not discussed here. You will be referred to a hematologist (a specialist in blood diseases) or an allergist by your dermatologist for further evaluation.

Self-care:

• Avoid wasp and bee stings.
• Inform all your doctors that you have mastocytosis. Carry a medication passport.
• Do not take just any medication. Show your medication passport or consult with your dermatologist or hematologist if you are unsure whether you can take a certain medication.