febrile neutrophilic skin disease
Introduction
Introduction to thermal neutrophil dermatosis Thermal neutrophil skin disease, known as Sweet's syndrome, also known as acute febrile neutrophilic dermatosis or inflammatory erythema, is rare in rheumatism and is mainly found in women. It is characterized by: 1 sudden onset of painful erythema nodules or plaques, mainly distributed in the arms, face and neck. 2 Histologically, the dermis showed characteristic mature neutrophil infiltration. 3 often have fever, systemic consumption and peripheral blood neutrophils. 4 lesions usually disappear after receiving glucocorticoid treatment, leaving no traces, but often repeated attacks. basic knowledge The proportion of illness: 0.003% Susceptible people: mainly seen in women Mode of infection: non-infectious Complications: Leukemia Multiple myeloma Diabetes Psoriasis
Cause
Cause of thermal neutrophil dermatosis
(1) Causes of the disease
The cause is unknown, and the predisposing factors are:
1. 5 to 7 days before onset, there are often upper respiratory tract infections, sore throat, cough, asthma, mouth ulcers or flu-like symptoms.
2. The skin lesions are aggravated after trauma or vaccination.
3. Some cases are associated with leukemia or other malignant tumors.
4. It is related to external stimuli such as sun exposure.
(two) pathogenesis
1. The pathogenesis may have three kinds of diseases: an allergic disease after infection. Some patients may give the skin intradermal injection of bacterial antigen (Streptococcus mutans) or fungal antigen (Candida albicans). Loss reproduction, its clinical and histopathological changes are the same as the original skin damage, it is speculated that bacteria or fungi are antigens, combined with the corresponding antibodies to form soluble immune complexes to activate complement, attract neutrophil accumulation, cause blood vessels and surrounding tissues Inflammation. 2 The relationship with malignant tumors, the incidence of leukemia in Sweet syndrome is nearly 10%, rash appears before and after the onset of leukemia, can also be a manifestation of leukemia recurrence, in addition to gastric cancer, cervical cancer, testicular embryo cancer A malignant tumor is considered to be a non-specific reaction caused by malignant cells as an antigen. 3 The disease occurs in the summer, and the rash is located at the exposed part, which is thought to be related to the sun and high temperature.
2. The pathological dermis shows dense mature neutrophil infiltration. The typical infiltrating foci are scattered spots or nodules, and the size is not more than 1mm. It is characteristically distributed in the upper 1/3 to 1/2 of the upper layer of the dermis. Occasionally, it can be found in the deep and subcutaneous tissues of the dermis. In addition to scattered spots and nodules, there is a rare and special inflammatory infiltration, which is in the form of continuous bands of varying degrees, distributed near the epidermis, sometimes neutrophils. Cell infiltration is fusion or diffuse, distributed throughout the dermis, inflammatory cells are mainly neutrophils, but lymphocytes, small amounts of eosinophils and mast cells are also seen in the lesions, and in some cases, infiltration Nuclear fragments are often seen in the foci. These fragments appear to be intact lobules of neutrophils. In some cases, a linear nucleolar dust particle is seen. The cell body of lymphocytes is larger than normal, mainly located in the infiltrating area. Around the capillaries and venules, the capillaries near the infiltrating foci show swelling of the endothelium and edema of the wall, but no typical leukocyte destructive vasculitis, and the blood vessels in the infiltrating foci can be marked. For thrombosis and focal wall destruction, changes in the epidermis are often mild, mostly showing mild hyperplasia of the spine cells, and other changes can occur in a few cases, such as focal cavernous changes, intraepithelial neutrophil infiltration , spongy small pustules, spotted keratosis and small segmental scaly molting, etc., even the formation of vacuoles.
In many cases, there may be obvious edema of the dermal papilla. This change causes the epidermis to bulge from the dermis to form a similar change in bullous skin disease. The typical feature is that dense collagen in the dermal papilla extends into the pseudo bullae. In the cavity, the cavity contains dilated capillaries, a small amount of exuded red blood cells and a different number of neutrophils. When the cavity is densely concentrated in the dermal papilla, it is similar to that of herpes dermatitis. Nipple abscess.
Prevention
Thermal neutrophil skin disease prevention
1. Eliminate and reduce or avoid the disease factors, improve the living environment, develop good living habits, prevent infection, pay attention to food hygiene, and rational diet.
2. Pay attention to exercise, increase the body's ability to resist disease, do not over-fatigue, excessive consumption, quit smoking and alcohol, maintain balance, and overcome anxiety and tension.
3. Early detection and early diagnosis of primary disease, other symptoms should be considered whether it is related to malignant tumors, establish confidence in the fight against disease, adhere to treatment.
4. Prevent excessive sun exposure in the exposed parts of summer, high temperature and high temperature environment.
Complication
Thermal neutrophil cutaneous disease complications Complications leukemia multiple myeloma diabetes psoriasis
Some patients may develop leukemia or pre-leukemia leukemia, leukemia may be chronic myeloid leukemia, acute granulocyte or granulocyte, monocytic leukemia, usually occurs after the first lesion occurs, or simultaneously Occurrence, skin lesions form blisters, ulcers, necrosis rate is higher, and systemic symptoms are heavier, hormone therapy is better, but easy to repeat, in addition to leukemia, but also with testicular embryonic cell carcinoma, visceral adenocarcinoma and Malignant tumors such as multiple myeloma are complicated, and in addition, Sjogren's syndrome, tissue capsular cytoplasmosis, diabetes, psoriasis and osteoarthrosis can be complicated.
Symptom
Symptoms of thermal neutrophil dermatosis Common symptoms Sore throat leukocytosis Dry cough joint pain Skin minor trauma ie... Hyperthermia eczema papule mucosal damage
Early manifestations include cold-like prodromal symptoms such as fever, chest discomfort, sore throat, dry cough, muscle pain, general malaise and gastrointestinal symptoms. Other early symptoms include meningeal irritation, joint pain and appendicitis-like symptoms, 90%. The patient may have different degrees of fever before or during the onset of the disease, especially when the skin lesion begins to appear, which is mostly high fever, and the body temperature can reach 40 °C.
1. Skin manifestations rash often appears suddenly, is multiple, can expand to the surrounding within a few days to several weeks, often fused into a piece, skin damage can be papules, nodules, plaques, small pustules, small blisters or Purpura-like skin lesions, etc., typical skin lesions are yellow beans to palms, plaques or nodules of light red to dark red or brownish red ring-shaped ridges, with clear edges, round or oval, local pain and touch Pain, often located in the arms and face, cheeks, both sides of the distribution, but asymmetry, generally have local hard and fever, the surface of the skin lesions are not smooth, the center is red or normal, more advanced can have scales and Pigmentation, surrounded by high, hard mung bean is like a blister-like pimples cluster, that is, the so-called fake blisters, the appearance is translucent, mostly not broken, rarely ulcers, individual cases may appear bullous or secondary infection, Eczema-like changes and scarring often occur.
In most cases, several rashes can exist at the same time. In some cases, there is only one rash. In a few cases, the palmar or oral mucosal damage can still occur.
With the disappearance of the rash, fever, rash and systemic symptoms disappear, the rash begins to disappear from the center, and after 1 to 2 months, it can completely disappear, leaving no scars, only mild desquamation. And pigmentation.
2. About 30% of joints can develop polyarticular pain or polyarthritis, which is characterized by joint pain and swelling of the elbow, wrist, knee and ankle. It usually resolves after 2 weeks, and individual cases can show chronic intermittent. Paroxysmal non-abnormal peripheral arthritis, arthritis and skin lesions often occur simultaneously, synovial biopsy shows non-specific inflammation, and synovial examination shows a large number of neutrophils and large mononuclear cells.
3. Ocular manifestations of conjunctivitis, superficial scleritis, symptoms of fear of light, tearing, increased secretions and eye pain, eye symptoms often appear at the same time as skin lesions, skin lesions subsided, eye symptoms It also fades on its own.
Examine
Examination of thermal neutrophil skin disease
1. Blood routine All patients with simple Svet syndrome have peripheral blood leukocytes and neutrophils. In 80% of cases, the total number of white blood cells can reach above 10.0×109/L, and the total number of white blood cells in 10% of cases is (2.025.0). × 109 / L, the proportion of mature neutrophils is 57% ~ 94%, an average of 80%, individual cases may have nuclear left shift, red blood cells and hemoglobin are mostly normal, individual cases may have moderate anemia, complicated with leukemia The total number of white blood cells decreased, especially in the first time after the rash episode was found to be shorter than the time when the myelodysplastic abnormality was found, the decrease was more obvious, and the platelets and red blood cells were decreased to different extents.
2. ESR increased.
3. Urine routine appearance of mild proteinuria and microscopic hematuria.
4. Bone marrow images of bone marrow with leukemia showed characteristic hypertrophic changes in leukemia.
5. Immunological examination is generally found without immunological abnormalities.
Diagnosis
Diagnosis and diagnosis of thermal neutrophil dermatosis
Diagnostic criteria
Generally, according to the unique clinical manifestations of the disease, combined with histopathological changes, diagnosis can be made. Currently, the diagnostic criteria for Svet syndrome, 1 main conditions include: A. sudden tenderness or painful erythema or purple spot Block, nodule, B. There is obvious neutrophil infiltration in the dermis, and no leukocyte fragment vasculitis. The two conditions include: A. First fever or infection, B. fever, joint pain, Conjunctivitis or a potentially malignant disease, C. leukocytosis, D. effective for hormone therapy and antibiotic treatment, who can meet the two main conditions and meet at least 2 secondary conditions, can be sure of the diagnosis.
Differential diagnosis
1. Polymorphic erythema is a vasculitic disease, and its rash is pleomorphic. It often has typical rainbow-like damage. The mucosa is often affected at the same time. It is more common in adolescents. The symptoms are mainly itching and the tenderness is not obvious. Histopathology is subcutaneous blister, the epidermis is often necrotic, and the dermis is mainly infiltrated by lymphocytes. Generally, there is no inflammatory infiltration of diffuse neutrophils, and no nuclear debris.
2. Persistent inflammatory erythema is slow onset, no systemic symptoms, rash is dark or gray-white nodules or plaques, mostly occur in the vicinity of the ear and joints, showing a symmetric distribution, the course of disease can be as long as 5 to 10 years, after healing Scars are left, not sensitive to glucocorticoid therapy, and histopathology is vascular inflammatory changes.
3. Small intestine short circuit syndrome due to morbid obesity in patients with small intestine short circuit surgery, clinical and histopathologically similar skin lesions can occur in this disease, this disease is also more common in women, its typical rash is small pustules, surrounded by The erythema, generally less than 6mm in diameter, is mainly distributed in the lower limbs, and occurs in multiple batches. It occurs once every 4 to 6 weeks, usually accompanied by arthritis and joint pain. The histopathology of the lesion shows that the dermal papilla and reticular layers are diffuse. Sexual edema, dermal neutrophil infiltration with nuclear fragmentation, similar to that seen in Sweet's syndrome, immunofluorescence can be seen at the dermal epithelial junction with immunoglobulin deposition, indicating that the small intestine short circuit syndrome is contained The immune complex of bacterial antigen is caused by antibiotic treatment, but it is not effective for antibiotic treatment of Svet syndrome.
4. Leukemia-associated bullous pyoderma (atypical gangrenous pyoderma) granulocytic leukemia can occur in inflammatory lesions, typical skin lesions involving the face, limbs and trunk, beginning to show erythema and plaque, Later, there are bullae around the center, necrosis or ulceration in the center, which is similar to the disease in histopathology, but the patient has fever, peripheral blood neutrophils, and typical acute myeloid leukemia or acute granules, mononuclear Cellular leukemia or myelofibrosis changes, and lesions can disappear after treatment with moderate to high doses of glucocorticoids.
5. Other diseases need to exclude systemic lupus erythematosus, sarcoidosis, through clinical features, histopathological changes and related laboratory tests, it is easy to identify.
