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The etiology and pathogenesis of reticulohistiocytosis are not completely known. The possibility of a viral or bacterial infection is assumed (in the anamnesis of children, infections are often noted - tonsillitis, otitis media, sepsis, etc.). The steadily progressive course of the disease may indicate the neoplastic nature of the process. A. M. Abezgauz believed that the genetic mutation factor is of great importance in the pathogenesis of the disease. Symptoms of reticulohistiocytosis. The disease usually begins in infancy, sometimes even in the neonatal period, with a sudden rise in temperature to 40 �� C. Then the temperature curve acquires a hectic character, but sometimes it can be subfebrile. On the scalp, in the area of �6�7�6�7the shoulder blades, abdomen and chest, a lot of hemorrhagic scaly lichenoid papules and petechial elements appear.

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Some patients with Abt-Letterer-Siwe disease may have onychia and paronychia. Erosions and hemorrhages are often noted on the mucous membranes of the mouth and vulva.

In the center of the papules, necrosis occurs, followed by the formation of dotted scars; sometimes infiltrative-tumor formations are observed, leaving larger scars.

Characteristic features are pronounced polyadenopathy and hepatosplenomegaly. Otitis and mastoiditis are often noted, which are resistant to antibiotic therapy and surgical intervention. In most patients, the bones of fosamax pills, ribs, collarbones, shoulder blades, limbs, and pelvis are affected, which is accompanied by pain and swelling of the soft tissues.

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On radiographs, foci of destruction, osteoporosis, osteolysis, decalcification zones of a cystic nature, periostitis are determined. There may be changes in the lungs of an infiltrative, small-focal and emphysematous nature, which are less often detected physically, but mainly radiologically (increased basal pattern, formation of cystic cavities, thickening of the walls of the bronchi, the presence of emphysema vesicles).

The general condition of the child deteriorates rather quickly, appetite disappears, the skin becomes pale, poor weight gain, weakness are noted.

In the peripheral blood: hypochromic anemia with aniso- and poikilocytosis, leukopenia or leukocytosis with neutrophilia and a left shift in the leukocyte formula to myelocytes; some patients have monocytosis. The myelogram is characterized by polymorphism of bone marrow cells, sometimes the content of reticulohistiocytic elements is increased.

Telemedicine service Histopathology: in the skin foci, clusters of histiocytes are found, among which there are lymphocytes and eosinophils. The protoplasm of some histiocytes is foamy, stained with fat. The epidermis can be destroyed due to the pressure of the underlying skin layers. In the visceral organs, normal structures are replaced by a proliferate of large pale reticulohistiocytic cells.

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