OVERVIEW
Recurrent cutaneous necrotizing eosinophilic vasculitis is a cutaneous vasculitis characterized by generalized red papules, hemorrhagic papules, and windswept plaques, with occasional annular erythema, edematous lesions, and blisters, which may be associated with angioedema, oral mucositis, gingivitis, and alopecia areata.
Etiology
The etiology of this disease is not clear. The pathogenesis is that eosinophils release IL-5, C4, platelet activating factor, etc. under the chemotaxis of vascular endothelial cell adhesion factor, integrins and intercellular adhesion factor, which increases vascular permeability and ultimately leads to the pathological changes of necrotizing vasculitis.
Symptoms
Clinical manifestations include generalized red papules, hemorrhagic papules, angioedema, rheumatoid plaques, mucositis, gingivitis, and alopecia areata. Occasionally, there are annular erythema, edematous lesions and blisters. Conscious itching, no systemic symptoms. The course of the disease is long, with a chronic relapsing process.
Histopathology
Pathology is mainly characterized by necrotizing vasculitis of small dermal vessels. The walls of the tubes have fibrin-like necrosis, and the whole dermis is infiltrated with eosinophils. There is mild or no leukocyte fragmentation.
Examination
1. Absolute eosinophil count is increased, blood sedimentation is accelerated, major basic protein (MBP) is increased in serum (normal value <600 μg/L), IgE, IgA and IgM are increased, and α and γ globulin are increased in serum protein electrophoresis. Eosinophils have prolonged survival time in the patient’s serum.
2. Histopathology: Pathology is mainly characterized by necrotizing vasculitis of small dermal vessels. There is fibrin-like necrosis in the walls of the tubes and eosinophilic infiltration throughout the dermis. There is mild or no leukocyte fragmentation.
Diagnosis
Combined with clinical manifestations and corresponding examination, the diagnosis is not difficult.
Treatment
Glucocorticosteroids have a good effect and can also be combined with immunosuppressive therapy. Topical topical glucocorticosteroids, combined with antihistamine therapy may result in mild improvement.