Erythema persistent towering: causes, symptoms, diagnosis, treatment

Erythema persistent exalted is considered as a localized form of leukocytoclastic vasculitis, although the site of this disease is not definitively determined. Distinguish the symptomatic form associated with taking medications and various diseases (ulcerative colitis, infection, polyarthritis), and a much rarer idiopathic form. Presume a possible relationship between persistent elevated erythema and paraproteinemia. To the variant of persistent erecting erythema, J. Herzberg (1980) classifies Urbach's extracellular cholesterosis. Single family cases are described. The clinically persistent erecting erythema manifests itself in pinkish or reddish-cyanotic nodules that are often clustered near the joints (especially on the dorsal surface of the hands, in the knee and elbow joints) and merging into large plaques of irregular, sometimes annular, contours, often with a westing in the center, that gives them a resemblance to the annular granuloma. Possible ulceration, bullous and hemorrhagic eruptions, with infection - the development of vegetation. The place of regressed foci is hyperpigmentation. Skin lesions of the type of persistent upright erythema may be observed in the Sweet syndrome.

Pathomorphology is characterized by signs of allergic vasculitis, i.e. Destructive changes in the walls of the vessels and their infiltration, as well as infiltration of the perivascular tissue mainly by neutrophilic granulocytes with the phenomena of karyorexis. In small vessels of the upper third of the dermis, swelling of endotheliocytes, fibrinoid changes, especially in fresh cells, and infiltration by neutrophilic granulocytes are noted. In older elements, the vessels undergo hyalinosis, which is characteristic of this disease. In the infiltrate, in addition to neutrophilic granulocytes, lymphocytes and histiocytes are found. Further, fibrotic changes in the vessels and dermis predominate, however, inflammatory infiltrates of a focal character can also be detected here, among which the leukoclasis is determined locally. There may be a different amount of extracellular deposits of cholesterol, which is associated with the yellow coloration of some lesions. With a significant deposition of cholesterol, a special variant of this disease is distinguished: extracellular cholesterosis, which is not associated with lipid metabolism disorders, but, probably, is the result of severe tissue damage with extra- and intracellular deposits of cholesterol.

Histogenesis. Most patients have circulating immune complexes, an increase in IgG or IgM, in 50% of cases a neutrophil chemotaxis defect is observed. Using the method of direct immunofluorescence using the immunoperoxidase method, lesions of immunoglobulins G, A, M and C3 of complement components around the vessels are revealed in the lesions. Some patients suffer from IgA or IgG monoclonal gammapathy or myeloma. The role of infectious agents, in particular streptococcal antigens and E. Coli in the development of the disease, is assumed.

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