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Mastocytosis


Mastocytosis is a group of scarce disorders common in both children and adults. In very rare cases, mastocytosis can affect other aread of the body, such as the stomach, the intestines and the bone marrow. Mast cell are present throughout the body, but are specially plentiful in the skin and digestive tract. Mast cells are part of the immune defense system. It is caused by the attendance of too many mast cells in your body. Mastocytosis is featurized by mast cell proliferation and accumulation within various organs, most commonly the skin. There are two kinds of mastocytosis: cutaneous (skin) and systemic.

There are unprecedented types of cutaneous and systemic forms. The most common cutaneous form is named as urticaria pigmentosa. The more severe form of the disease is known as Systemic Mastocytosis (SM), meaning the mast cells are increased in other areas of the body. The medical symptoms and signs of systemic mastocytosis are due to the deposition of these clonally derived mast cells in different tissues, involving bone marrow, skin, the GI tract, the liver, and the spleen.

In children, who have mastocytosis only in the skin, it is most probably which it will go away. Mastocytosis influences males and females equally. Most reported cases are in whites. The cutaneous lesions of most types of mastocytosis are less visible in more excessively pigmented skin. Mast cells play an essential role in helping your immune system vindicate these tissues from disease. Mast cells make a chemical called histamine. Frequently, this chemical serves as a kind of alarm to let the immune system know which an infection is invading part of the body.

Types of cutaneous mastocytosis involve solitary mastocytoma (M), diffuse erythrodermic mastocytosis, paucicellular mastocytosis, and urticaria pigmentosa (UP). More invasive forms of mastocytosis and mast cell leukemias are very rarely confronted. Urticaria pigmentosa can be diagnosed by the abnormally high concentration of mast cells in the skin. Rarely, urticaria pigmentosa advances to systemic mastocytosis during adulthood. In children, a mastocytoma usually disappears voluntarily.

Causes of Mastocytosis

The common causes and risk factor's of Mastocytosis include the following:

  • The presence of too many mast cells in a person's body.
  • Symptoms may be focussed by cold or heat, certain medicines, emotional stress and insect bites.
  • Mastocytosis likely is a hyperplastic reaction to an abnormal stimulus.
  • A genetic problem has been present in some patients.
  • When mast cells infleunce organs such as the liver, spleen, lymph nodes, and bone marrow.
  • Underlying medical situations are other medical conditions which may probably cause Mastocytosis.

Symptoms of Mastocytosis

Some sign and symptoms related to Mastocytosis are as follows:

  • Itching.
  • Abdominal cramping.
  • Red, itchy rash.
  • Bone pain.
  • Muscle pain.
  • Nausea and vomiting.
  • Stomach ulcers.
  • Rash that looks like freckles.

Treatment of Mastocytosis

Here is list of the methods for treating Mastocytosis:

  • Topical steroids transiently reduce skin lesions that are cosmetically disturbing.
  • Corticosteroids can be used topically, inhaled, or systemically to reduce inflammation linked with mastocytosis.
  • Antihistamines which work particularly against ulcers and proton pump inhibitors relieve ulcer-like symptoms.
  • Cromolyn (disodium cromoglycate) is a mast cell stabilizing drug which is frequently effective in reducing skin and gastrointestinal symptoms as well as mental and other systemic systems.
  • Ultraviolet light and corticosteroid creams applied to the skin may be used to cure the skin symptoms of mastocytosis.
  • Immunotherapy may rarely be advantageous to select patients with mastocytosis.