Mastocytosis (Urticaria Pigmentosa)
Description
Mastocytosis is caused by an abnormal growth of mast cells. Mast cells are anti-inflammatory cells made in the bone marrow and they release the chemical histamine. Histamine causes swelling, itching and redness and is one of the substances responsible for an allergic reaction. This disorder can occur at any age but is most often seen in children. Many times the disease will resolve on its own in puberty. The most common site for the increased number of mast cells is in the skin although other areas such as the intestines can be affected. The symptoms are caused by a trigger such as exposure to cold or heat, certain medications, insect stings, or emotional stress. Symptoms can be isolated to the skin or can involve the entire body and at times be life threatening.
Symptoms
Skin symptoms include a red itchy rash, hives, red to brown blotches that resembles freckles and skin swelling after itching. Abdominal pain and diarrhea can occur. Some patients can have what looks like a life threatening allergic reaction consisting of fainting, sweating, confusion, low blood pressure and breathing problems.
Tests
A history and physical exam will be done. A skin biopsy and/or a urine test for histamine may be performed.
Treatment
Skin symptoms are treated with anitihistamines and over the counter steroid creams. Patients with severe reactions should have a medical alert bracelet and carry a self injectable epinephrine pen. Patients should try to avoid any triggers that cause their symptoms. Some patients have problems with general anesthesia and they should notify their provider of their condition before any medical procedure.
Related Videos
Research
- Allergy and Immunology
- Dermatology
- Family Practice
- Internal Medicine
- Pediatric Allergy and Immunology
- Pediatric Dermatology
- Pediatrics
Share Your Experience
Do you have experience with this disease? Do you or someone
you know suffer from Mastocytosis (Urticaria pigmentosa)?
Please share you experience with other members of the iTriage community.
I have been diagnosed with Systemic Mastocytosis. I have been diagnosed to be in the low spectrum of Phase 1. I have cutaneous spots all over my chest, back, inner arms, upper and lower legs. I find wearing under armor shirts to help a great deal rather than cotton shirts. I am a 53 year old male in Florida. I was diagnosed in March 2010. I'm not under going any treatment at this time as its been a long time since I've had any flareups. Email me if you would like to discuss this.