Necrotizing urticarial vasculitis: causes, symptoms, diagnosis, treatment

Necrotizing urticaro-like vasculitis can occur in chronic idiopathic urticaria, with systemic diseases (systemic lupus erythematosus) and other effects. For the first time this type of vasculitis was identified by FC McDuffic et al. (1973) among patients with chronic urticaria. Later, this disease was called "urticariform vasculitis."

Skin manifestations clinically resemble an idiopathic urticar rash, which must be taken into account in differential diagnosis. Among the usual urticaria are more dense and larger flat foci, existing from 24 to 72 hours. Sometimes in the center of the foci, spot hemorrhages are seen than they differ from the rash in the usual urticaria. After the disappearance of purple foci, slight pigmentation remains. Rare clinical options are bullous lesions, reminiscent of multiforme exudative erythema. The rash is often accompanied by itching, and in some patients there are soreness of the foci, abdominal pain, increased ESR. With systemic lesions, arthralgia, lymphadenopathy, myositis, and less often diffuse glomerulonephritis are observed. Based on the clinical picture, WP Sanchez et al. (1982) share urticaroid-like vasculitis in two ways: with hypocomplexemia and normo-complementation. Most patients with hypocomplexemia have arthritis and abdominal pain. In patients with normocomplamenia, systemic lesions, hematuria and proteinuria are revealed.

Pathomorphology of necrotizing urticaro-like vasculitis. Discover a classic picture of necrotizing leukoclastic vasculitis. Characteristic features are fibrinoid necrosis of the vascular walls, mainly venules, in combination with endotheliocytic hyperplasia of certain 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 leukoclasis, especially pronounced with hypocomplexemia. There are cases with sharply expressed tissue infiltration by eosinophilic granulocytes, whose nuclei have signs of karyorexis and degranulation. In a state of degranulation, there are also tissue basophils. In large foci, besides the vessels of the superficial plexus, the inflammatory phenomena of the character described above are also observed in the deep venous plexus.

Histogenesis of necrotizing urticaro-like vasculitis. The results of the reaction of direct immunofluorescence in the affected skin are quite variable. First of all, deposits of IgG and IgM, as well as C3-components of complement, less often IgA, are found around the vessels. In the region of the basement membrane, deposits of IgG and C3 components of complement are more common, and the granular deposits of IgM are less often chalky.

McDuffic et al. (1973), this type of vasculitis revealed immune complexes in the majority of patients, which is why it is attributed to immunocomplex diseases. Often, especially in severe cases, hypocompleteemia 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. In the blood, antinuclear antibodies and rheumatoid factor in most cases do not show.

Differentiate from chronic idiopathic urticaria, systemic lupus erythematosus, mixed cryoglobulinemia of IgG and IgM types and congenital angioedema.

[1], [2], [3]