Mastocytosis

Mastocytosis is an abnormal accumulation of mast cells in the skin and sometimes in various other parts of the body. Located in skin, the linings of the stomach and intestine, as well as other sites, mast cells may play an important role in helping defend these tissues from disease. Mastocytosis develops when mast cells increase in number and accumulate in tissues over a period of years. Mast cells, a component of the immune system, produce histamine, a substance involved in allergic reactions and the production of stomach acid. Because the number of mast cells increases, levels of histamine increase. They are also found around wounds, and seem to play a role in healing of injuries. 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 (UP), which occurs when mast cells infiltrate the skin. Systemic mastocytosis is caused by the accumulation of mast cells in the tissues and can affect organs such as the liver, spleen, bone marrow, and small intestine. Urticaria pigmentosa was first described in 1869, while systemic mastocytosis was first reported in the scientific literature in 1933. The true incidence of either type of mastocytosis remains unknown, but mastocytosis generally is considered to be an "orphan disease."

The most common form of mastocytosis (say "mass-toe-sigh-toe-sis") is a disease of the skin. Mast cells contain many different natural chemicals, a common one being histamine. The most common cutaneous (skin) form is also called urticaria pigmentosa, which occurs when mast cells infiltrate the skin. In a rare form, mast cells accumulate as a single mass in the skin (mastocytoma). In a form called urticaria pigmentosa, mast cells accumulate in many areas of the skin, forming small reddish brown spots or bumps. Rarely, urticaria pigmentosa progresses to systemic mastocytosis during adulthood. In systemic mastocytosis, mast cells accumulate in the skin, stomach, intestines, liver, spleen, lymph nodes, and bones.

Causes of Mastocytosis

The common Causes of Mastocytosis :

Underlying medical conditions are other medical conditions that may possibly cause Mastocytosis.
It is not known what causes mastocytosis, but a genetic problem has been found in some patients
It is not contagious, i.e. can't be caught by other people in contact with your child.
Mastocytosis probably is a hyperplastic response to an abnormal stimulus.
We do know some things that trigger the release of histamine from mast cells and cause the symptoms of mastocytosis.

Symptoms of Mastocytosis

Some Symptoms of Mastocytosis :

Abdominal discomfort .
Diarrhea.
Nausea and vomiting .
Stomach ulcers.
Skin lesions.
Episodes of very low blood pressure and faintness .

Treatment of Mastocytosis

Several medicines help treat the symptoms of mastocytosis.

Antihistamines to work against mast cell chemicals
Medicines to relieve cramping in the intestines
Antihistamines block receptors targeted by histamine released from mast cells. Both H1 and H2 blockers may be helpful.
Corticosteroids can be used topically, inhaled, or systemically to reduce inflammation associated with mastocytosis.
Leukotriene antagonists block receptors targeted by leukotrienes released from mast cells.
Immunotherapy (Allergy Shots) may rarely be beneficial to select patients with mastocytosis. The benefits of the shots should be weighed against the substantially increased risk of severe and potentially fatal anaphylaxis triggered by the shots themselves.
Proton pump inhibitors help reduce production of gastric acid, which is often increased in patients with mastocytosis. Excess gastric acid can harm the stomach, esophagus, and small intestine.

Nails Care

Anychia
Clawing of the Nails
Clubbing
Discoloration of the Nail Plate
Ingrowing Toe Nail
Koilonychia
Longitudinal Striations
Loss of Nail Plate
Onycholysis
Paronychia
Pitting of Nail Plates
Pterygium
Thickening of Nail Plate
Transverse Ridges

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