The girl to your left is one-year old Stephanie Brown from Mirfield, West Yorkshire in England who suffers from Diffuse Cutaneous Mastocytosis or DCM. This is a disease where itchy spots and hives can appear on one’s skin. This non-catagious condition is the result of the body producing too many of a particular type of red blood cell called mast cells (aka mastocytes).
Mast cells can be found in skin, linings of the stomach and intestine, and connective tissue (such as cartilage or tendons). Mast cells play an important role in helping your immune system defend these tissues from disease. Mast cells have also been found to gather around wounds and may play a part in wound healing. For example, the typical itching you feel around a healing scab may be caused by histamine released by mast cells. Researchers also think mast cells may have a role in the growth of blood vessels.
The presence of too many mast cells, or mastocytosis, can occur in two forms-cutaneous and systemic. The most common cutaneous (skin) form is also called urticaria pigmentosa, which occurs when mast cells infiltrate the skin. Systemic mastocytosis is caused by mast cells accumulating in the tissues and can affect organs such as the liver, spleen, bone marrow, and small intestine. Diagnosis of cutaneous mastocytosis is normally through skin biopsies performed by physicians. When a doctors detects a large number of mast cells on the skin, the patient is confirmed to have the disorder. Systemic mastocytosis is mastocytosis is confirmed when an unusually large number of mast cells are found in the bone marrow.
Mastocytosis can take on two forms-cutaneous and systemic. The most common cutaneous (skin) form is also called urticaria pigmentosa, which occurs when mast cells infiltrate the skin. Systemic mastocytosis is caused by mast cells accumulating in the tissues and can affect organs such as the liver, spleen, bone marrow, and small intestine.
Symptoms
Chemicals released by mast cells cause changes in your body’s functioning that lead to typical allergic responses such as flushing, itching, abdominal cramping, and even shock. When too many mast cells are in your body, the additional chemicals can cause:
- Musculoskeletal pain
- Abdominal discomfort
- Nausea and vomiting
- Ulcers
- Diarrhea
- Skin lesions
It can also cause episodes of hypotension (very low blood pressure and faintness) or anaphylaxis (shock).
Treatment
Mastocytosis is treated through the use of a number of different medicines, including antihistamines (to prevent the effect of mast cell histamine) and anticholinergics (to relieve intestinal cramping). A number of medicines treat specific symptoms of mastocytosis.
- Antihistamines frequently treat itching and other skin complaints
- Antihistamines that work specifically against ulcers and proton pump inhibitors relieve ulcer-like symptoms
Two types of antihistamines treat severe flushing and low blood pressure before symptoms appear and epinephrine after symptoms begin:
- Topical steroids temporarily reduce skin lesions that are cosmetically disturbing
- Steroids treat malabsorption, or impaired ability to take in nutrients
In cases in which mastocytosis is malignant, cancerous, or associated with a blood disorder, steroids and/or chemotherapy may be necessary.
Sources:
2.bp.blogspot.com
Cleveland Clinic
