What Is Sweet's Syndrome?

Acute febrile neutrophilic skin disease, or Sweet syndrome, is a rare disease and is mainly seen in women. It is characterized by: sudden painful erythema nodules or plaques appearing mainly on the arms, face, and neck; in Histologically, the dermis shows characteristic mature neutrophil infiltration; often has fever, systemic exhaustion, and increased neutrophils in peripheral blood; skin lesions usually disappear after treatment with glucocorticoids, leaving no trace But often recurrent. The syndrome is generally an idiopathic minority of cases, which can be complicated by myeloproliferative diseases, such as granulocytes or granulocytic leukemia, and individual cases can be complicated by other malignant diseases.

Basic Information

nickname

Sweet syndrome, Sweet disease

English alias

Sweet Syndrome

Visiting department

dermatology

Multiple groups

female

Common locations

Arm, face and neck skin

Common causes

Infections, drugs, tumors, skin trauma, etc.

Common symptoms

Fever, chest discomfort, sore throat, dry cough, muscle pain, general discomfort

Causes of Acute Fever Neutrophil

The etiology is unknown, and its predisposing factors are:

Infection

5 to 7 days before the onset of symptoms such as sore throat, cough, asthma, oral ulcers or flu-like symptoms of upper respiratory tract infections.

2. Drugs

Granulocyte colony-stimulating factor, all-trans retinoic acid, minocycline, trimethoprim-sulfamethoxazole, carbamazepine, hydralazine, and contraceptives

3. tumor

Some cases are accompanied by leukemia or other malignancies, such as acute myelogenous leukemia, lymphoma, and anemia or erythrocytosis. About 15% of malignant tumors are solid tumors, such as tumors of urogenital tract, breast, and gastrointestinal tract. The skin disease can be the initial manifestation of a malignancy, or months or years before the diagnosis of the malignancy. Repeated episodes suggest a recurrence of underlying tumors.

4. Associated with other diseases

Behcet syndrome, nodular erythema, sarcoidosis, rheumatoid arthritis, and thyroid disease.

5. Skin trauma

Possible mechanisms are related to hypersensitivity to bacteria, viruses, drugs, tumor antigens, etc. and local and systemic cytokine secretion disorders, including interleukin 1, granulocyte colony-stimulating factor, granulocyte-macrophage colony-stimulating factor and interferon Wait.

Clinical manifestations of acute febrile neutrophil

Early manifestations are cold-like precursor 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% of patients can have fever to varying degrees before or during the onset of disease, especially when skin lesions begin to appear, most of which are hyperthermia, and the body temperature can reach 40 ° C.

Skin manifestation

The rash often appears suddenly, is multiple, and can expand and increase in the surroundings within a few days to weeks, often fused into a film. Skin damage can be pimples, nodules, plaques, small pustules, small blister, or purpuric skin lesions. Typical skin lesions are soybeans to large palms, which are light red to dark red or brown-red raised plaques or nodules, with clear edges, round or oval shapes, and local pain and tenderness. It is often located on the arms, face, and cheeks, and is bilaterally distributed, but asymmetric. Generally, there are localized stiffness and fever, and the surface of the lesion is not smooth, and the center is red or normal. There may be scales and pigmentation at a later stage, and there are hard, mung beans around the blister-like papules, which are called pseudo-blister. The appearance is translucent, most of them are not ulcerated, and ulcers are rarely formed. Individual cases may have bullae or secondary infections, often with eczema-like changes and scabs.

2. Joint performance

Polyarthritis or polyarthritis can occur in about 30% of cases, manifested as pain and swelling of joints such as elbows, wrists, knees, and ankles, which usually subside after 2 weeks. Individual cases can show chronic intermittent non-deformity Peripheral arthritis, arthritis, and skin lesions often occur simultaneously. Synovial biopsy showed non-specific inflammation, and synovial fluid examination showed a large number of neutrophils and large monocytes.

3. Eye performance

Presented as superficial scleritis of conjunctivitis. The conscious symptoms include eye symptoms such as fear of light, tears, increased secretions, and eye pain, which often occur at the same time as the skin lesions, which disappear, and the eye symptoms also subside.

Examination of acute febrile neutrophil

Blood routine

All patients with simple type of Sweet Syndrome have increased peripheral white blood cells and neutrophils. The total number of white blood cells in 80% of cases can be above 10.0 × 10 ⁹ / L, and the total number of white blood cells in 10% of cases is (2.0 25.0) × 10 ⁹ / L, the proportion of mature neutrophils ranged from 57% to 94%, with an average of 80%. Individual cases may have more than moderate anemia, and patients with leukemia, the total number of white blood cells decreased, especially the shorter the interval between the first rash and the discovery of abnormal bone marrow hyperplasia, the decline is more obvious, and platelet and red blood cells have decreased to varying degrees .

2. Erythrocyte sedimentation rate

3. Urine routine

Urinary mild proteinuria and microscopic hematuria

4. Myeloid

Bone marrow findings in patients with leukemia show characteristic leukemia hyperplasia.

5. Immunological examination

Generally no immunological abnormalities were found.

Diagnosis of acute febrile neutrophil

Generally based on the unique clinical manifestations of the disease, combined with histopathological changes, a diagnosis can be made. The current diagnostic criteria for Sweet Syndrome: The main conditions include: A. Sudden tenderness or painful erythema or mauve plaques, nodules. B. There is obvious neutrophil infiltration in the dermis without leukocyte fragmentation vasculitis. Minor conditions include: A. Fever or infection first. B. Fever, joint pain, conjunctivitis, or potentially malignant disease. C. Leukopenia. D. Effective for hormonal therapy but ineffective for antibiotic therapy. Anyone who meets two major conditions and at least two minor conditions can be surely diagnosed.

Treatment of acute febrile neutrophil

1. Glucocorticoid is effective in treating this disease. Prednisone is available at the beginning. Most patients can resolve endothelial damage within 3 to 1 week, and systemic symptoms such as fever, pain, and arthritis disappear within 2 to 3 days. Glucocorticoids should be gradually reduced after 4 to 6 weeks.

2. Dapsone treatment, even for 2 to 4 weeks, can also achieve better results.

3. In addition to the above treatments, if potassium iodide is used for 2 weeks, good results can also be obtained. Can also be used indomethacin, continuous medication for 2 to 4 weeks, to control the symptoms of the acute phase.

Glucocorticoids are used in combination with non-hormonal drugs. For example, prednisone plus potassium iodide is used for 2 consecutive weeks. The effect is outstanding and it is not easy to relapse.

4. Treatment of complications For patients with leukemia or other diseases, in addition to the above treatment methods, appropriate combined chemotherapy for combined leukemia or other malignant tumors and systemic supportive therapy should be given. Appropriate symptomatic treatment should be performed for patients with Sjogren's syndrome, such as the use of hypoglycemic drugs to treat diabetes and cortisone eye drops to treat eye symptoms.