Lipomeningocele retikulez

Lipomeningocele retikulez (synonym: reticulosishyperplastica lipomelanotica Pautrier et Woringer, lymphadenitis dermatopathica, lymphadenopathia lipomelanotica) - nonspecific hyperplastic reaction simpaticas nodes on some chronic generalized exfoliative zudaschie medicine. This reaction is an expression disorders exchange between the epidermis and the dermis, skin and lymphatic system. Superficial lymph nodes in the context of yellowish brown and black specks. Their histological structure preserved. In the cortical substance follicles - large germinal centers, in the pulp - sharp hyperplasia reticular cells hemosiderin, melanin and fat inclusions in the cytoplasm. Among cellular elements lot of eosinophils, meet neutrophils, plasma, sometimes large cells, similar to the cells Berezovsky - Sternberg. In the interim sinus - congestion reticular cells. The skin changes corresponding to one or another form of dermatosis. In the basal layer cells endothelium and in chromatophores from a lot of melanin, where it penetrates into the lymph nodes, which struck again. Reticulohistiocytosis hyperplasia and pigmentation lymph nodes develop simultaneously, but independently of one another.
Often affects men. The skin is a chronic (very rarely acute), common, itchy (rarely without itching) erythematous-squamous medicine (for example, eritrodermii some hematogenic, red flat versicolor, psoriasis, chronic eczema, erythrodermic stage of mycosis fungoides, malignant chlamydia, and others). Lymph nodes, especially inguinal (sometimes tracheo-bronchial), enlarged, firm, painless, not soldered. Sometimes an enlarged liver, spleen. In blood moderate leukocytosis, polioles, eosinophilia to 33%; can be hyperproteinemia with alpha - and gamma globulinemia. Rash when lipoplasties retikuleze can be secondary, due to diseases such as mushroom avium, follicular retikulez, Hodgkin's disease, leukemia and other
For further diagnosis lipoplasties of reticulata a histological examination of lymph nodes. In malignant chlamydia find cell Berezovsky - Sternberg, the proliferation of fibroblasts; mushroom avium violated the basic histological structure of the lymph nodes, no phagocytic activity reticular cells; follicular lymphoma drastically changed structure of the lymph follicles more, they are more diverse, often separated by gaps; when metastases melanoblastoma has a primary focus, rapid generalization of the process.
Forecast serious; in 3-5 years killed about half of the patients.
Reliable methods of treatment there. Used long-term high doses of corticosteroids, periodically - injection of ACTH; local - x-ray treatments.