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Lipomelanotic reticulosis

Lipomelanotic reticulosis (synonym: reticulosishyperplastica lipomelanotica Pautrier et Woringer, lymphadenitis dermatopathica, lymphadenopathia lipomelanotica) - non-specific hyperplastic lymph node reaction to some chronic generalized exfoliative pruritic dermatoses. This reaction is an expression of metabolic disorders between the epidermis and the dermis, skin and lymphatic system. Superficial lymph nodes on a section yellowish with brown and black specks. Their histological structure is preserved. In the cortical substance of the follicles there are large germinal centers, in the pulp there is a sharp hyperplasia of the reticular cells with hemosiderin, melanin and fatty inclusions in the protoplasm. Among the cellular elements of many eosinophils, there are neutrophils, plasma, sometimes large cells, similar to the cells of Berezovsky - Sternberg. In the intermediate sinuses - clusters of reticular cells. In the skin - the changes corresponding to one or another form of dermatosis. In the basal layer, endothelial cells and in chromatophores there is a lot of melanin, from where it penetrates the lymph nodes, which are secondarily affected. Reticulohistiocytic hyperplasia and pigmentation of the lymph nodes develop simultaneously, but independently of one another.

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More often men are ill. On the skin - chronic (very rarely acute), widespread, itchy (rarely without itching) erythematous-squamous dermatoses (for example, erythroderma in some hematodermas, lichen planus, psoriasis, chronic eczema, erythrodermic stage of fungal mycosis, malignant lymphocytosis, chronic lymphocytosis, chronic eczema, erythrodermic stage of fungal mycosis, malignant lymphocytosis, chronic lymphocytosis, chronic eczema, erythrodermic stage of fungal mycosis, malignant lymphocytosis, chronic lymphoma, malignant lymphocytosis, chronic erectile dysfunction . Lymph nodes, especially inguinal (sometimes tracheo-bronchial), are enlarged, dense, painless, not soldered. Sometimes enlarged liver, spleen. In the blood, moderate leukocytosis, polynucleosis, eosinophilia up to 33%; there may be hyperproteinemia with alpha and gamma globulinemia. Rash with lipomelanotic reticulosis can be secondary, caused by diseases such as fungal mycosis, follicular reticulosis, Hodgkin's disease, leukemia, etc.

To clarify the diagnosis of lipomelanotic reticulosis, histological examination of lymph nodes is necessary. In malignant lymphogranulomatosis cells are found Berezovsky - Sternberg, the proliferation of fibroblasts; in fungal mycosis, the main histological structure of the lymph nodes is disturbed, there is no phagocytic activity of the reticular cells; in follicular lymphoma, the structure of the lymph nodes is drastically changed, the follicles are larger, they are more diverse, often separated by slits; with melanoblastoma metastases, there is a primary focus, rapid generalization of the process is noted.

The prognosis is serious; in 3-5 years about half of the patients die.

There are no reliable methods of treatment. Long-term large doses of corticosteroid drugs are used, periodically - injections of ACTH; Local - radiotherapy.