Sweet Syndrome ( Acute Febrile Neutrophilic Dermatosis) is a rare disorder characterized by fever and the sudden onset of a rash, which consists of multiple tender, red or bluish-red bumps or lesions. These lesions usually occur on the arms, legs, trunk, face or neck. In some cases, additional systems of the body can become involved including the musculoskeletal system such as inflammation of the joints (arthritis), the eyes such as inflammation of the conjunctiva or the membrane that lines the eyes (conjunctivitis), and the internal organs. In the majority of affected individuals, the disorder occurs by itself for no known reason (idiopathic Sweet syndrome); this is also known as classical Sweet syndrome.
Subdivisions of Sweet Syndrome
- Classical Sweet Syndrome
- Malignancy-associated Sweet Syndrome (MASS)
- Drug-induced Sweet Syndrome
Signs & Symptoms
The major symptom of Sweet syndrome is the sudden onset of tender or painful bumps (nodules or papules) on the arms, legs, face or neck. They may also occur on the thighs and trunk. Papules are solid, raises lesions; nodules are slightly larger and may extend deeper into the skin. These initial lesions are usually several millimeters to centimeters in diameter, but sometimes up to an inch in diameter, flat or slightly elevated, irregularly-shaped, and inflamed. They tend to grow slowly, eventually joining together (coalescing) to form larger, irregular plaques. Small pus-filled blisters (pustules) may develop.
In some individuals, neutrophils may accumulated in the fatty layer of tissue just below the skin (subcutaneous fat) rather than in the dermis. Affected individuals often develop reddish (erythematous) discoloration of the skin and small bumps (nodules) on the skin. The arms and legs are most often affected.
A diagnosis of Sweet syndrome is made based upon a thorough clinical evaluation, a detailed patient history, identification of classic symptoms, and a variety of specialized tests. In many cases, surgical removal (biopsy) and microscopic examination of small samples of skin tissue may reveal the infiltrate of neutrophils in the dermis. A complete blood cell count may also show neutrophils in the blood (neutrophilia).
The treatment of Sweet syndrome is directed toward the specific symptoms that are apparent in each individual. In some cases, Sweet syndrome may resolve itself with no treatment, although this can take weeks to months. The mainstay of treatment is with systemic corticosteroids. In most cases, treatment with low doses of corticosteroids such as methylprednisolone or prednisone has proven effective in eliminating symptoms, sometimes rapidly resolving symptoms. However, Sweet syndrome often recurs periodically despite therapy. For isolated lesions, local therapy may consist of topical corticosteroids (creams of gels) or directly injecting corticosteroids into the lesion (intralesional corticosteroid).
Other drugs have been used to treat individuals with Sweet syndrome including colchicine, dapsone, and potassium iodide. These drugs are generally used for individuals who cannot tolerate corticosteroids, in whom corticosteroids were ineffective, or when trying to lower the dose of (taper) the corticosteroid. A variety of additional drugs have been used to treat individuals with Sweet syndrome including cyclosporine, indomethacin and clofazimine.
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