Nodular panarteritis: causes, symptoms, diagnosis, treatment

Nodular panarteritis (syn.: nodular panvasculitis, nodular periarteritis, Kussmaul-Meyer disease, necrotizing angiitis) is a systemic disease caused by vascular damage, probably of autoimmune genesis, which is confirmed by the detection of immune complexes in the walls of the affected vessels. In the classic (systemic) variant of the disease, the process mainly involves the vessels of the internal organs. Skin changes are observed in approximately 25% of patients.

Skin lesions in the systemic form of nodular panarteritis are characterized by polymorphism of the rash, consisting mainly of purpuric elements and erythematous spots. Among them, blisters and necrotic changes with ulceration may be observed, which resembles the picture of leukoclastic vasculitis. In addition, there are foci of livedo racemoza, less often - cutaneous-subcutaneous nodular elements. There is a so-called cutaneous form of nodular panvasculitis, which is limited only to changes in the skin vessels, although N.E. Yarygin (1980) considers it as the initial phase of the disease, which over time acquires a generalized character. With this form, fever, myalgia or arthralgia may be observed. Clinically, this form, although characterized by polymorphism of the rash, is less pronounced than the systemic form, there is a smaller tendency to the development of necrotic changes. The most characteristic symptoms are painful nodular rashes, most often located on the extremities, less often on other areas of the skin.

Pathomorphology of nodular panarteritis. Arteries of medium and small caliber are predominantly affected. However, studies by N.E. Yarygin et al. (1980) show that vessels of all links of the microcirculatory bed are involved in the process, which indicates a systemic lesion. Depending on the ratio of alterative, exudative and proliferative changes, arteritis can be destructive, destructive-productive and productive. Nodular panarteritis is characterized by polymorphism of the histological picture, reflecting the chronic course of the process and the change in phases of strengthening and weakening of immune disorders in the body. In this regard, in the presence of acute destructive, destructive-proliferative and proliferative changes, sclerosis of the arteries with signs of exacerbation of the process is noted.

In the skin and subcutaneous tissue, the systemic form of nodular panarteritis mainly affects muscular vessels. The vessels are affected segmentally, which corresponds to nodular formations on the skin. The systemic form is characterized by leukoclastic vasculitis with fibrinoid necrosis of the vessel walls, pronounced infiltration of both the walls themselves and the perivascular tissue by lymphocytes, neutrophilic and eosinophilic granulocytes, the nuclei of which often undergo karyorrhexis with the formation of "nuclear dust". Thrombosis is sometimes noted. Along with acute manifestations, chronic changes are observed in the form of swelling and proliferation of endothelial cells, sclerosis, which sometimes leads to closure of the lumen of the vessel; in the tissue surrounding the vessels - fibrosis. A characteristic feature is the combination of acute and chronic changes: foci of mucoid swelling can be observed in fibrously altered tissue, and in the acute stage - fibrinoid necrosis.

In the cutaneous form of nodular panarteritis, the histological picture is similar to that in the systemic form: acute changes of the leukoclastic vasculitis type and chronic ones are detected, characterized by sclerosis of the vessel walls with the closure of their lumens, as a result of which ulcers can often form. The infiltrate located around the vessels contains lymphocytes and macrophages.

Histogenesis of nodular panarteritis. Direct immunofluorescence in the lesions often reveals deposits of IgM or C3 complement component in the walls of the affected vessels, and less often they are found together. These deposits are located mainly in small vessels of the superficial dermis network and very rarely in its deep parts.

In the development of the disease in adults, antigens such as hepatitis B virus and cryoglobulins are important. In recent years, an association with HIV infection has been observed. In childhood, vasculitis mainly develops against the background of infection with group A streptococci.

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