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Introduction:
Acute febrile neutrophilic dermatosis, commonly known as Sweet’s syndrome, is a rare skin condition characterized by the sudden onset of fever, elevated neutrophil counts, and painful skin lesions. It was first described by Dr. Robert Douglas Sweet in 1964. The condition can be idiopathic or associated with other underlying diseases such as infections, malignancies, or autoimmune disorders.
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Clinical Features:
A. Symptoms:
1. Fever: High-grade fever is often one of the first symptoms.
2. Skin Lesions: Painful, red or purple plaques and nodules, primarily on the face, neck, upper limbs, and trunk.
3. Malaise: General feeling of discomfort or illness.
4. Muscle and Joint Pain: Arthralgia and myalgia can accompany skin manifestations.
5. Ocular Involvement: In some cases, patients may experience conjunctivitis or episcleritis.
B. Signs:
– Lesion Characteristics: The skin lesions are typically well-demarcated and can become pustular or bullous.
– Laboratory Findings: Elevated neutrophil counts (neutrophilia) are a hallmark, along with elevated inflammatory markers like ESR (erythrocyte sedimentation rate) and CRP (C-reactive protein).
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C. Pathogenesis:
The exact cause of Sweet’s syndrome is not entirely understood, but it is believed to be an abnormal immune response. The condition is associated with increased levels of cytokines, which lead to neutrophil activation and migration to the skin. Several triggers have been identified, including:
– Infections: Bacterial, viral, or fungal infections can precipitate the condition.
– Malignancies: Hematologic cancers like leukemia and solid tumors.
– Medications: Certain drugs, including granulocyte colony-stimulating factor (G-CSF), can induce Sweet’s syndrome.
– Autoimmune Diseases: Conditions such as rheumatoid arthritis and inflammatory bowel disease.
D. Diagnosis:
The diagnosis of Sweet’s syndrome is primarily clinical, supported by laboratory and histopathological findings. The diagnostic criteria include:
1. Major Criteria:
– Abrupt onset of painful erythematous plaques or nodules.
– Histopathologic evidence of dense neutrophilic infiltration without leukocytoclastic vasculitis.
2. Minor Criteria (two or more required):
– Preceding fever or infection.
– Association with an underlying malignancy or inflammatory disease.
– Response to corticosteroids.
– Elevated inflammatory markers.
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E. Differential Diagnosis:
Several conditions can mimic Sweet’s syndrome, making differential diagnosis crucial:
– Infections: Cellulitis, erysipelas.
– Other Neutrophilic Dermatoses: Pyoderma gangrenosum, Behçet’s disease.
– Vasculitis: Cutaneous vasculitis, systemic lupus erythematosus.
– Drug Reactions: Drug-induced hypersensitivity reactions.
F. Treatment:
The primary treatment for Sweet’s syndrome is systemic corticosteroids, which typically result in rapid improvement. Other treatments include:
1. Corticosteroids:
Prednisone is the most commonly used, with doses tapering based on clinical response.
2. Nonsteroidal Immunomodulators:
– Colchicine
– Dapsone
– Potassium iodide
3. Immunosuppressive Agents:
Used in refractory cases.
– Cyclosporine
– Azathioprine
– Methotrexate
4. Targeted Biological Therapies:
In cases associated with malignancies or autoimmune diseases, biologics like TNF inhibitors may be used.
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G. Prognosis:
The prognosis for Sweet’s syndrome is generally good, especially with prompt treatment. However, the condition can recur, particularly in cases associated with malignancies or chronic inflammatory diseases. Regular follow-up and monitoring for underlying conditions are essential.
Conclusion:
Acute febrile neutrophilic dermatosis, or Sweet’s syndrome, is a complex condition that requires careful clinical evaluation and management. Understanding its clinical features, pathogenesis, and treatment options is crucial for effective management. While it is a rare disease, awareness among clinicians can lead to prompt diagnosis and treatment, improving patient outcomes and quality of life.