Mastocytosis in children
Venereology / / August 12, 2017
Mastocytosis - a relatively rare disease caused by the proliferation of mast cells in various organs and tissues.The frequency of mastocytosis in children is 1: 1400-1: 2500 visits to primary children of dermatological institutions.
For decades cutaneous mastocytosis described called urticaria pigmentosa.On the basis of the predominance of mast cells (mast cells) to infiltrate urticaria pigmentosa it was proposed to call this disease mastocytosis.Mastocytosis refers to benign retikulezah in which the majority of patients with a primary process is a skin lesion.
mastocytosis often affects children, although the disease occurs at any age.Often the first signs of the disease are detected at birth.
Etiopathogenesis disease remains completely unclear.It is believed that this disease nevoid character promote disease development toxic effects, toxicity, liver and gastrointestinal tract.It is believed that the disease can be genetically determined as described familial diseases, including twins.
There are cutaneous and systemic form of the disease.Adrift mastocytosis may be benign or malignant.If systemic mastocytosis, which is extremely rare in children, along with the skin lesions of the liver is involved in the process, spleen, bone marrow, lymph nodes, and so on. D.
Numerous clinical varieties of cutaneous mastocytosis.In most cases the disease manifests itself in the form of spots and nodules of round or oval shape, brownish, yellowish or brownish-yellow color, not scaly.The process is localized on the scalp, face, trunk and extremities.The palms and soles, and mucous membranes are affected very rarely.Much less frequently observed knotted mastocytosis in small patches of yellowish-brown or yellowish color with a smooth or wrinkled surface.This kind of clinical cutaneous mastocytosis may show single or multiple nodules, often prone to merge in places of folds.
Approximately 10-15% of patients with the disease manifests itself in the form of a mastocytoma (tumor clinical kind), which usually occurs at birth or in early childhood.Clinically, it is often solitary mastocytoma spot nodule or plaque with a diameter of 2 cm or more, brown or brownish-yellow color, with the injury of (friction or mechanical impact) on the surface of bubbles appear.A rare clinical variant of the disease is diffuse mastocytosis, where the skin becomes rough, thickened, testovatoy to the touch, often accompanied by severe itching.It should be noted that the majority of children 2-3 years of age for all clinical varieties mastocytosis after mechanical injury blisters appear, disappear after a few days without a trace.
general condition of patients usually is not broken.Mostly the disease is not accompanied by subjective disorders or they appear after friction, taking a hot bath.In extremely rare cases, symptoms may occur due to the release in the tissue and blood from the fat cells of biologically active substances, especially gietamina: bouts of itching, redness (flushing), tachycardia, abdominal pain, fever, headaches.These effects usually last from 3-5 to 30 minutes and then pass spontaneously.It is also pertinent to note that these phenomena occur periodically, with age disappear completely.The most important pathognomonic symptom is a positive symptom mastocytosis Unna - property vysypnyh elements after rubbing swell, take on the appearance of blisters and acquire a bright red color.
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Systemic mastocytosis in children is extremely rare.There may be enlargement of the liver, spleen, lymph nodes, gastrointestinal tract.Bone lesions usually revealed by X-ray examination in the form of a limited osteosclerosis or osteoporosis.Extremely rare is the malignant form of systemic mastocytosis - mast cell leukemia - a poor prognosis for life.
prognosis of mastocytosis in children is favorable.In most patients, the disease undergoes spontaneous regression at the end of adolescence.In order to avoid systemic mastocytosis, all patients must undergo a thorough clinical and laboratory examination (clinical blood and urine tests, a study of the functional state of the liver and kidneys, X-rays of the bones and gastrointestinal tract).
Treatment of patients with mastocytosis is a difficult task.Note the positive therapeutic effect of the combined use of antibradikininovyh and antihistamines (diphenhydramine, diazolin, tavegil, Phencarolum) hystoglobulin.Encouraging results in the application of zaditetsa.