Mastocytosis

Mastocytosis (mastocytosis; from German. Mast - fattening and Greek. kytos - cell - a system of skin-visceral syndrome caused by the proliferation of fat cells. Mastocytosis considered as navodno disease, genodermatoses or as reticuloendotheliosis. In the dermis - dense infiltration fat cells, diffuse or perivascular. The size of cells 12-18 MK, kernel extended, sometimes multiple, with rare chromatin and 1 - 2 nucleoli. In the cytoplasm - metachromatic grains of different sizes. In the surface part of the dermis they scattered like dust.
The same infiltration may be some internal organs, especially in the bone marrow and spleen. Fat cells are physiological geparinoidam; this explains it happens sometimes in patients with mastocytosis hemorrhages and blood clotting.
There are the following forms of mastocytosis: 1) PS - brownish the tumor with shagreen surface with a diameter of 5 cm, is confined to the extremities; 2) multiple hillocky; 3) multiple papular with soft lenticular papules (sometimes with lichenification); 4) bullous - very rare, when bubbles appear spontaneously, regardless of friction; 5) skin and diffuse (masticatory retikulez). At last the skin is thickened or pasty, sometimes lichenification, cream-colored, with some elevations, as shagreen; in great folds - depth grooves, is a giant soft lichenification, erythroderma with areas of swelling on areas of friction and skin pigmentation, urticaria, infiltration, as in the diffuse geotermia, sometimes izyaslavs tumors. For mastocytosis also include pigment urticaria (see) and mastocytoma - single hemispherical clearly distinguished tumor, often on the face.
When mastocytosis always celebrate severe itching attacks, red sublime dermographism, very easily called and keeping up to 30 minutes or more, without itching. In blood may be macrocytic and normohromnaja anemia with leukopenia and trombopenia, the presence of fat cells.
Describes the lymph nodes, liver, spleen, bone marrow, lung, pancreas, phenomena of osteoporosis. Such severe the lesions are usually few years lead to a lethal outcome. Mastocytosis should be differentiated from the nevus (see Naevus), xanthomatosis, reticulatum (see), gemidiriya. The prognosis is generally favorable.
Treatment. Individual elements can be removed surgically. At the widespread rash, sometimes fast and good, but temporary effect of radiotherapy fractional doses in 8 - 10 days, a total course of 100-500 R. General treatment - vitamins, corticosteroids.