Recurrent cutaneous necrotizing eosinophilic vasculitis

Introduction to recurrent skin necrotic eosinophilic vasculitis Recurrent cutaneous necrotic eosinophilic vasculitis (recurrentcutaneousnecrotizingeosinophilicvasuclitis) is a systemic red papule, hemorrhagic papules, angioedema, wheal plaques, mucositis, gingivitis, total baldness, etc., occasional annular erythema , edematous damage and cutaneous vasculitis of the blister. basic knowledge The proportion of illness: 0.03%--0.07% Susceptible people: no special people Mode of infection: non-infectious Complications: erythema

Causes of recurrent skin necrotic eosinophilic vasculitis

(1) Causes of the disease

The cause of this disease is not clear.

(two) pathogenesis

The pathogenesis is still not clear. Considering that eosinophils adhere to vascular endothelial cells, the presence of adhesion molecules can continuously supplement eosinophils. Eosinophils in vascular cells and cytokine-induced vascular cell adhesion molecules Type 1 (VCAM-1) combines integrin late active antigen type 4 (VLA-4) and intercellular adhesion molecule type 1 (ICAM-1) to play an important role in the migration of eosinophils to endothelial cells. The release of leukotriene from Cleavage cells, C4 and platelet-activated factors increase vascular permeability and lead to the pathological changes caused by the release of granule proteins and the release of histamine from basophils and mast cells. The pathology is mainly small in the dermis. Angiogenic vasculitis (Figure 1).

Pathology showed fibrin-like necrosis in the wall, eosinophil infiltration in the whole dermis, mild or leukocyte fragmentation, normal dermis, occasional epidermal blister or eosinophil infiltration, indirect immunofluorescence There are a large number of major basic proteins and neurotoxins derived from eosinophils deposited in the blood vessel wall and the blood vessels around the blood vessels, and it can be seen in the dermis, especially in the perivascular cells with mast cells (stained with mast cells trypsin), electron microscopy Examination showed that the small blood vessel wall and endothelial cells were abnormal, and there were adhesions of eosinophils and free eosinophils, and the damaged endothelial cells were visible. The eosinophils showed cytoplasmic granules and organelles lost, and the chromatin of the nucleus dissolved. Abnormal endothelial cells have nuclear pyknosis, cell swelling, mitochondrial destruction, cell membrane rupture, etc., there are degenerative large eosinophils and free particles between the collagen bundles, stained with immunoperoxidase, visible small blood vessel endothelial cells have VCAM -1 deposition, and VLA-4 positive large eosinophil adhesion, vascular superficial involvement of VCAM-1 is strongly positive.

Recurrent skin necrotizing eosinophilic vasculitis prevention

Early diagnosis, early treatment, as far as possible to find the cause, to treat the cause.

Recurrent skin necrotic eosinophilic vasculitis complications Complications

There may be superficial lymphadenopathy and hepatosplenomegaly.

Recurrent skin necrotic eosinophilic angiitis symptoms common symptoms hemorrhagic papules skin necrosis wheal full bald vascular allergic inflammation

The rash showed systemic red papules, hemorrhagic papules, angioedema, wheal plaques, mucositis, gingivitis, total baldness, occasional ring erythema, edema damage and blisters, main itching, no body Symptoms, long course of disease, a chronic recurrence process.

Examination of recurrent skin necrotic eosinophilic vasculitis

According to clinical manifestations, laboratory tests, tissue biopsy features can be diagnosed.

The absolute count of eosinophils increased, reaching (1.4 ~ 6.2) × 10 / L, erythrocyte sedimentation rate, serum major protein (MBP) increased (<600g / L), IgE, IgA, IgG increased, serum protein electrophoresis And gamma globulin increased, eosinophils prolonged survival in the patient's serum.

Diagnosis and diagnosis of recurrent skin necrotic eosinophilic vasculitis

Must be identified as herpes-like dermatitis, eosinophilia syndrome, Wells syndrome, episodes of angioedema with eosinophilia, polymorphic pregnancy rash, etc., the above diseases are clinically no hemorrhagic papules, pathology There are also significant differences, so it is not difficult to distinguish, Churg-Strauss syndrome, clinically asthma, histopathology is small to medium-sized vascular eosinophilic vasculitis, or granulomatous vasculitis, and there is no white blood cell fragmentation Can be identified with it.

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