Erythema persistent elevated: causes, symptoms, diagnosis, treatment

Erythema persistent elevated is considered a localized form of leukocytoclastic vasculitis, although the final location of this disease has not been determined. A symptomatic form associated with the use of drugs and various diseases (ulcerative colitis, infections, polyarthritis) and a much rarer idiopathic form are distinguished. A possible connection between persistent elevated erythema and paraproteinemia is assumed. J. Herzberg (1980) refers extracellular cholesterosis of Urbach to a variant of persistent elevated erythema. Isolated familial cases have been described. Clinically, persistent erythema protuberans is manifested by pinkish or reddish-blue nodules, often located in groups near the joints (especially on the back of the hands, in the area of the knee and elbow joints) and merging into large plaques of irregular, sometimes ring-shaped, outlines, often with a depression in the center, which makes them similar to annular granuloma. Ulcers, bullous and hemorrhagic rashes are possible, and in case of infection - the development of vegetation. Hyperpigmentation remains at the site of regressed foci. Skin lesions of the persistent erythema protuberans type can be observed in Sweet's syndrome.

Pathomorphology is characterized by signs of allergic vasculitis, i.e. destructive changes in the vessel walls and their infiltration, as well as infiltration of perivascular tissue mainly by neutrophilic granulocytes with karyorrhexis phenomena. In small vessels of the upper third of the dermis, swelling of endotheliocytes, fibrinoid changes are noted, especially in fresh elements, and their infiltration by neutrophilic granulocytes. In older elements, the vessels undergo hyalinosis, which is characteristic of this disease. In addition to neutrophilic granulocytes, lymphocytes and histiocytes are found in the infiltrate. Subsequently, fibrous changes in the vessels and dermis predominate, but even here one can find focal inflammatory infiltrates of varying intensity, among the cells of which leukoclasia is determined in places. Extracellular cholesterol deposits may be present in varying quantities, which is associated with the yellow color of some lesions. With significant cholesterol deposits, a special variant of this disease is distinguished - extracellular cholesterosis, which is not associated with lipid metabolism disorders, but is probably the result of severe tissue damage with extra- and intracellular cholesterol deposits.

Histogenesis. Most patients have circulating immune complexes, elevated IgG or IgM levels, and 50% of cases have a defect in neutrophil chemotaxis. Direct immunofluorescence with the immunoperoxidase method reveals deposits of immunoglobulins G, A, M, and C3 complement components around the vessels in the lesions. Some patients suffer from IgA or IgG monoclonal gammopathy or myeloma. Infectious agents, particularly streptococcal and E. coli antigens, are suspected of playing a role in the disease.

[ 1 ], [ 2 ], [ 3 ], [ 4 ], [ 5 ], [ 6 ]