What is it?

Sweet syndrome, also known as Acute febrile neutrophilic dermatosis, is a rare disorder characterized by fever,and an abrupt onset of a rash. The rash consists of numerous sensitive, red bumps or lesions. It usually affects arms, legs, trunk, face or neck. These patients may also present with arthritis or conjunctivitis. Classical Sweet Syndrome is idiopathic- for no known reason, usually after an upper respiratory tract or gastrointestinal infectionr Seldom, it is associated with malignancies, usually hematologic or secondary to drug use like GCSF.

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Additional names

This group contains additional names:
- Acute Febrile Neutrophilic Dermatosis
- granuloma annulare
- Other Erythematous Condition
- Acute erythema
- Danthron erythema
- Epidermal collarette
- Erythema elevatum diutinum
- Erythema fugax
- Erythema scarlatiniforme
- Excessive smegma
- Genitocrural intertrigo
- Granuloma annulare
- Intertrigo
- Liver palms
- Micropapular weal
- Pityriasis rubra
- Submammary intertrigo

Signs & symptoms

The main symptom of the syndrome is a sudden onset of painful papules (solid raised lesions) or nodules (larger and deeper lesions) affecting the legs, arms, neck or face. Their shape is irregular, they are flat or elevated and they usually grow, join together and may form bigger plaques. Other symptoms of Sweet syndrome may be fatigue, headaches, malaise, fever (may precede the cutaneous presentation) , arthritis or arthralgia. Eyes can be affected and present with conjunctivitis, episcleritis, limbal nodules, glaucoma, iritis and peripheral ulcerative keratitis. Those who have comorbidity with leukemia can suffer from oral mucositis.

Diagnosis

Diagnosis is usually based on clinical examination and anamnesis. skin biopsy will confirm the diagnosis if an inflammation that is composed mainly of neutrophils is present without vasculitis. On blood tests ESR, CRP and WBC might be increased, p-ANCA or c-ANCA are sometimes present

Treatment

Oral corticosteroids are an efficient treatment, resulting many times in quick clinical improvement. Those who cannot tolerate corticosteroids might use Other drugs including colchicine, dapsone, and potassium iodide, ciclosporin, Anakirna, infliximab, adalimumab, minocycline, mycophenolate, clofazimine and thalidomide.

☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.

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