Recurrent cutaneous necrotizing eosinophilic vasculitis is a cutaneous necrotising vasculitis characterized by generalized red papules, hemorrhagic papules, angioedema, wheals, mucositis, gingivitis, alopecia totalis, and occasionally erythema annulare, edematous lesions, and vesicles.
The cause is unknown. The pathogenesis is the chemotaxis and adhesion of eosinophils under the action of endothelial leukocyte adhesion molecule 1 (ELAM-1), integrin VLA-4, and intercellular adhesion molecule 1 (ICAM-1). Eosinophils release leukotrienes, C4, and platelet activating factor, increasing vascular permeability, leading to vascular pathological changes.
Signs and Symptoms
Generalized red papules or purplish red hemorrhagic papules, wheals, and occasionally erythema annulare, edematous lesions, and vesicles, probably with angioedema, oral mucositis, gingivitis, and alopecia totalis, are present. Subjective symptoms are pruritus. Superficial lymphadenopathy and hepatolienomegaly are present. Systemic symptoms and systemic involvement are absent. The disease has a chronic recurrent process.
The absolute count of eosinophils is (1.4 - 6.2) × 109/L, erythrocyte sedimentation rate is elevated, α and γ globulin in serum protein electrophoresis are increased, and IgE, IgA, IgM are increased.
The main manifestation is necrotizing vasculitis of small vessels in the dermis. Fibrinoid necrosis on the vascular wall and eosinophil infiltration with mild or no leukocytoclasis in the whole dermis can be seen. The epidermis is generally normal, and occasionally vesicles or eosinophil infiltration in the epidermis are visible.
On the basis of clinical manifestations and histopathology, the disease can be diagnosed
Glucocorticoids are effective, and immunosuppressive agents can be used as well.