Skin histiocytosis X: causes, symptoms, diagnosis, treatment

Histiocytosis X (syn: histiocytic medullary reticulosis, malignant reticulogistiocytosis). Histiocytosis from Langerhans cells constitute a group that includes the Letterter-Sieve disease, Hend-Schüller-Kriscen's disease, eosinophilic granuloma, congenital self-healing reticulogistiocytosis, and histiocytosis from nondeterministic cells.

A rare progressive systemic disease of the mononuclear-histiocytic system with neoplastic proliferation of atypical histiocytes and other mononuclear phagocytes. The course of the disease is acute with an average life expectancy of up to 6 months. In this case, hepatosplenomegaly, bone marrow infiltration, lymphadenopathy, jaundice, fever, anemia are noted. N. Rappaport (1966) distinguishes two clinical forms of malignant histiocytosis: a visceral form with early involvement of hemopoietic organs and a cutaneous form, observed in approximately 10% of cases.

In the cutaneous form, clinically plaque-poor erythema or multiple papules from brownish to violet with a tendency to: hemorrhages and ulceration, subcutaneous nodes reminiscent of those with erythema nodosum, purple, sometimes observed in early contractions of the disease.

Pathomorphology. In the skin there is mainly a focal polymorphic infiltrate localized in this way perivascularly and perifolikulyarno. The more May infiltrate occupies the deep sections of the dermis and the subcutaneous fatty tissue. Cytological signs vary greatly. Distinguish the following groups of proliferating cells; atypical neoplastic histiocytes - large with puyryrus or hyperchromic, often lobular nuclei containing nucleoli, and basophil pyroninophilic cytoplasm; macrophages with nuclei of various configurations, containing in their cytoplasm numerous erythrocytes as a consequence of erythrophagocytosis, cellular detritus, platelets, hemosiderin and lipid-containing complexes; well-differentiated histiocytes are large oval or elongated cells with a zosinophilic cytoplasm and a vesicle containing phagocytic erythrocytes and other inclusions. Among the above forms, there are numerous cells of a transitional type. Necrosis is often observed. In addition to histiocytes, infiltrates contain neutrophilic and eosinophilic granulocytes, plasma cells, lymphocytes, giant cells, often bizarre forms resembling reticulosarcoma cells. Small non-phagocytic cells with pale cytoplasm and hyperchromic nuclei are identical with so-called pro-gistocytes, although their structure is not clearly defined. In subcutaneous adipose tissue, a picture of panniculitis with focal lipogranulomas is often noted. Histochemically, in many cells, the activity of nonspecific esterase is determined diffusely or in the form of granules. Ultrastructurally, histiocytes reveal specific formations in the form of "rackets", which can serve as a reliable diagnostic marker of histiocytosis X.

[1], [2], [3]