Mastocytosis

Urticaria Pigmentosa Urticaria pigmentosa is a specific childhood form of mastocytosis, in which there are pink-brown spots on the skin due to abnormal colletions of mast cells. It should not be confused with other, more severe, adult forms of mastocytosis. Mast cells are normally present in the skin. They contain granules that contain histamine and other chemicals. When the mast cell is disturbed these chemicals are released into the surrounding skin. The chemicals make the blood vessels leaky, resulting in localized itching, swelling, redness (a hive-like reaction). Clinical Features Urticaria pigmentosa most often affects infants, with the first patches appearing a few months of age, or even at birth. They may be confused with insect bites or cafe au lait birthmarks at first. They presist and gradually increase in number for several months or years. They can apprear on any part of the body including the scalp, face, trunk and limbs. In ulticaria pgmentosa, one can demonstrate the presence of mast cells by rubbing one of the brown patches. Within a few minutes, the rubbed area becomes red, swollen, itchy, and may even blister. This is known as Dariers sign, and confirms the diagnosis of urticaria pigmentosa. In young children, it is common for the patches to blister when rubbed. If many patches are activated at the same time the infant may become itchy and irritable, but it is uncommon for severe symptoms to arise. Over the next few years the skin lesions become less irritable and eventually the patches fade away. By the teenage years, most patches will have gone. Sometimes urticaria pigmentosa developes for the first time in a teenager or an adult. Children with more extensive or reactive skin lesions may experience facial flushing, diarrhea, or abdominal pain. Precautions Exercise, heat, or friction can aggravate symptoms. A severe reaction can result in flushing and faintness. Certain medications can cause mast cell degranulation and should be avoided if there is extensive urticaria pigmentosa. These include: Aspirin (salicylates) Codeine and morphine (narcotics) Alcohol Polymyxin cough medicines containing alcohol or codiene Tests Urticaria pigmentosa is generally so characteristic that no specific tests are necessary. However, occasionally a skin biopsy is needed to confirm the diagnosis. If there is presistent flushing, faintness, or diarrhea, the following tests may be helpful: Urinary histamine levels or 1.4-methylimidazole acetic acide (may be elevated) Treatment Urticaria pigmentosa is not serious, and does not require any treatment in most cases. However the following can be helpful. Oral antihistamines Mast cell stabilizers Disodium cromoglycate orally may be helpful, especially for presistent gastrointestinal symptoms. Topical Steroids Potent steroid creams applied for several weeks under occlusion can reduce itching and unsightliness, but the patches tend to recur within a few months. Topical steroids are only suitable for limited areas and for limited periods of time.

Mastocytosis What is mastocytosis? Mastocytosis is a group of disorders in both children and adults caused by the presence of too many kmast cells in a person's body. Mast cells are located in skin, the linings of the stomach and intestine, as well as other sites and may play an important role in helping defend these tissues from disease. By releasing chemicals such as histamine, mast cells attract other key players of the immune defense system to areas of the boyd where they are needed. Histamine is involved in reactions such as allergies and hives. There are several different forms of cutaneous mastocytosis. Some are cutaneous (skin) and others are systemic (involve internal body organs). Cutaneous (skin only) mastocytosis is the most common form. It is also called uticaria pigmentosa (if there are several lesions) or solitary mastocytoma (if there is a single lesion). CM mostly affects children. Diffuse cutaneous mastocytosis is a rare familial (inherited) form of mastocytosis in which all of the skin is affected. These childhood forms of mastocytosis should NOT be confused with the more severe adult forms. Systemic mastocytosis is caused when mast cells collect in the tissues and can affect organs such as the liver, spleen, lymph nodes, and bone marrow. This tends to occur in adults and is rare in children. What are the symptoms of childhood mastocytosis? Chemicals released by mast cells cause hove or allergy symptoms including: Itching, Abdominal Cramping, Diarrhea, Facial Flushing, Skin lesions which swell, blister, or become red, Wheezing (less common), and Episodes of low blood pressure and faintness (uncommon). How is childhood mastocytosis diagnosed? Doctors can often diagnose these conditions by seeing the characteristic lesions which are light pik-brown to yellow-brown in color, stay in the same location (do not come and go) and become red or swollen when rubbed. A small skin sample (biopsy) is sometimes needed to confirm the diagnosis. How is mastocytosis treated? Several medicines help treat the symptoms of mastocytosis. Antihistamines (such as Benadryl) to help with itching or skin reactions. Medicines (such as cromolyn sodium) to relieve presistent abdominal cramping or diarrhea.

I have this information on my web page because it is an extremely rare skin condition that my 5 month old happens to have. Took a lot to get this diagnosed as it is so rare! She is currently under the care of a pediatric dermatologist at Chrildren's Hospital in Dallas, TX.