Nodular panarteritis: causes, symptoms, diagnosis, treatment

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

The cutaneous lesions in the systemic form of the knobby panarteritis are characterized by the polymorphism of the rashes, consisting mainly of purpurotic elements and erythematous spots. Among them, there may be blisters and necrotic changes with ulceration, which resembles the picture of leukoclastic vasculitis. In addition, there are foci of livedo racemoza, rarely - skin-subcutaneous nodular elements. There is a so-called cutaneous form of gnarled panvasculitis, which is limited only by changes in the vessels of the skin, although N.E. Yarygin (1980) regards it as the initial phase of the disease, acquiring a generalized character over time. With this form, fever, myalgia or arthralgia can be observed. Clinically, although this form is characterized by polymorphism of the rash, but less pronounced than the systemic one, there is a lesser tendency to develop necrotic changes. The most characteristic for her are painful nodular-nodular eruptions, located more often on the limbs, less often in other parts of the skin.

Pathomorphology of the knobby panarteritis. Most arteries of medium and small caliber are affected. However, N.E. Yarygin et al. (1980) show that the vessels involve all the links of the microcirculatory channel, which indicates the systemic nature of the lesion. Depending on the ratio of alterative, exudative and proliferative changes, arteritis can be destructive, destructive-productive and productive. Nodular panarritis is characterized by polymorphism of the histological pattern, reflecting the chronic course of the process and the change in the phases of amplification and weakening of immune disorders in the body. In this regard, in the presence of acute destructive, destructive-proliferative and proliferative changes, sclerosis of arteries with signs of aggravation of the process is noted.

In the skin and subcutaneous tissue in the systemic form of nodular panarteritis, mainly muscle-type vessels are affected. 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 and perivascular tissue with lymphocytes, neutrophilic and eosinophilic granulocytes, the nuclei of which are often subjected to karyorexis with the formation of "nuclear dust". Sometimes there is thrombosis. Along with acute manifestations, there are chronic changes in the form of swelling and proliferation of endothelial cells, sclerosis, which sometimes leads to the closure of the lumen of the vessel; in the surrounding tissue vessels - fibrosis. Characteristic is the combination of acute and chronic changes: in fibro-altered tissue can be observed foci of mummy swelling, and in the acute stage - fibrinoid necrosis.

In the cutaneous form of the nodular panarteritis, the histological pattern is analogous to that in the systemic form: they reveal acute changes in the type of leukoclastic vasculitis and chronic sclerosis of the vessel walls with closure of their lumens, which can often lead to the formation of ulcers. Infiltrate, located around the vessels, contains lymphocytes and macrophages.

Histogenesis of nodular panarteritis. With direct immunofluorescence in the lesion foci, deposits of the IgM or C3 complement component in the walls of the affected vessels show up more often, they are found together more rarely. These deposits are located mainly in small vessels of the superficial network of the dermis and very rarely in its deep sections.

In the development of the disease, adults are given importance to such antigens as hepatitis B virus and cryoglobulins. In recent years, there has been an association with HIV infection. In childhood, vasculitis predominantly develops against the background of infection with group A streptococci.

[1], [2], [3], [4]