Necrotizing urticarial-like vasculitis: causes, symptoms, diagnosis, treatment

Necrotizing urticarial-like vasculitis may occur in chronic idiopathic urticaria, in systemic diseases (systemic lupus erythematosus) and other influences. This type of vasculitis was first identified by FC McDuffic et al. (1973) among patients with chronic urticaria. Later, this disease was called "urticarial-like vasculitis".

Skin manifestations clinically resemble idiopathic urticarial rash, which must be taken into account in differential diagnostics. Among the usual urticarial foci, there are denser and larger flat foci that exist from 24 to 72 hours. Sometimes, punctate hemorrhages are visible in the center of the foci, which distinguishes them from the rash with ordinary urticaria. After the disappearance of purpuric foci, slight pigmentation remains. Rare clinical variants are bullous lesions resembling erythema multiforme exudative. The rash is often accompanied by itching, and some patients experience soreness of the foci, abdominal pain, and increased ESR. In systemic lesions, arthralgia, lymphadenopathies, myositis, and less often, diffuse glomerulonephritis are observed. Based on the clinical picture, WP Sanchez et al. (1982) divide urticaria-like vasculitis into two types: with hypocomplementemia and normo-complementemia. Most patients with hypocomplementemia have arthritis and abdominal pain, while patients with normo-complementemia have systemic lesions, hematuria, and proteinuria.

Pathomorphology of necrotic urticarial-like vasculitis. A classic picture of necrotic leukoclastic vasculitis is revealed. Characteristic signs are fibrinoid necrosis of the vascular walls, mainly venules, in combination with hyperplasia of endothelial cells of some vessels and perivascular hemorrhages. Around the vessels there are infiltrates of various sizes, consisting of lymphocytes with an admixture of a large number of neutrophilic granulocytes with phenomena of leukoclasia, especially pronounced in hypocomplementemia. There are cases with a sharply expressed tissue infiltration by eosinophilic granulocytes, the nuclei of which have signs of karyorrhexis and degranulation. Tissue basophils are also in a state of degranulation. In large foci, in addition to the vessels of the superficial plexus, inflammatory phenomena of the above-described nature are also observed in the deep venous plexus.

Histogenesis of necrotic urticarial-like vasculitis. The results of the direct immunofluorescence reaction in the affected skin are quite variable. Around the vessels, deposits of IgG and IgM are found, as well as the C3 component of complement, less often IgA. In the zone of the basement membrane, deposits of IgG and the C3 component of complement are more common, and finely granular deposits of IgM are less common.

McDuffic et al. (1973) found immune complexes in most patients with this type of vasculitis, which is why it is classified as an immune complex disease. Often, especially in severe cases, hypocomplementemia is observed due to a decrease in total hemolytic complement, individual factors of the complement cascade (Clq, C4, C2, C3), while factors C5 and C9 are not changed. Antinuclear antibodies and rheumatoid factor are not detected in the blood in most cases.

It is differentiated from chronic idiopathic urticaria, systemic lupus erythematosus, mixed cryoglobulinemia of IgG and IgM types, and hereditary angioedema.

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