Mastocytosis

Mastocytosis is a chronic disease that affects the skin, as well as internal organs, bones. It is based on pathological processes caused by the accumulation and proliferation of mast cells (or lambrocites, mast cells) and the release of biologically active substances from them. The most frequent varieties of the disease are spotted and papular mastocytosis, often combined with each other and known as pigmentary urticaria, which is characterized by widespread and abundant cyanotic-pink, later brownish-brown spots and papules of small size and rounded outlines. Rashes occur usually as a type of multiple attacks with short remissions. The flow is uncertain. Pigmental urticaria, which occurred in early childhood, especially in the thoracic, to the onset of puberty, is often subjected to spontaneous regression. In adults, it is characterized by an increasing severity.

Less often, mastocytosis is manifested by nodes ranging in size from a pea to a bean, usually a brownish-yellowish coloration, often merging, especially in large folds, into extensive conglomerates; Bubbles that occur, as a rule, in early childhood and most often overlap with other variants of this disease; Diffuse foci with clear boundaries, with the increase in size and number of which erythroderma can form; A mastocytoma resembling a histiocytoma or non-carcinoma; Persistent spotted telangiectasia in the form of various sizes and outlines of spots consisting of densely intertwining telangiectasias on a pigmented background. Skin lesions can be joined by lesions of internal organs (especially liver and spleen), peripheral blood and bone marrow and other common disorders with possible fatal outcome.

Eruptions in friction, pressure, thermal procedures and other irritants become bright red, swollen and itchy (Darje-Unna phenomenon). Diagnosis always requires histological confirmation.

Treatment. Effective therapy is absent. Assign antihistamines (fenkarol, diazolin, suprastin, tavegil and especially zaditen), conduct photochemotherapy.