A clinical case of histiocytosis X, rarely found in the practice of a dermatovenereologist, is described, the pathognomonic clinical symptoms of this dermatosis, including skin lesions, that occur in 50 to 80 % of cases of all forms are displayed. The modern classification of histiocytosis, pathogenetic mechanisms of development and clinic of histiocytosis Х are given. Particular attention is paid to diagnostic methods, the necessity of interaction of narrow specialists (dermatovenerologists, hematologists, oncologists, pulmonologists) is underlined.
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