Sweets syndrome acute febrile dermatosis A

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rare condition described by Dr. R. D. Sweet in 1964, consisting of fever, a high white blood cell count, and a plaquelike skin rash. Sweet's syndrome is more common in women than men. Sweet's syndrome can cause arthritis and can be mistaken for still's disease or vasculitis.

Cause

The cause of Sweet's syndrome is not known. It is associated with some cancers, particularly leukemia, infections, drugs such as lithium, connective tissue diseases such as RA and SLE, and inflammatory bowel disease, suggesting that an abnormal immune response is responsible. Approximately 20 percent of patients with Sweet's syndrome have an underlying malignancy, usually affecting the bone marrow.

Symptoms

The characteristic symptoms of Sweet's syndrome are fever and rash. Fever and other systemic symptoms such as arthralgia, arthritis, myalgia, and conjunctivitis occur in most patients. Arthritis affects large joints such as knees, hips, wrists, and ankles but can also affect the fingers.

The rash of Sweet's syndrome usually consists of red plaques that are often tender. The plaques can be large, up to several inches in diameter, and can occur anywhere, including the face, arms, and trunk. Lesions on the legs can be mistaken for erythema nodosum, which can occur with Sweet's syndrome.

Diagnosis

The diagnosis of Sweet's syndrome is usually based on the appearance of the rash and a skin biopsy that shows dense infiltrates of neutrophils around blood vessels but no vasculitis. Laboratory tests usually show an elevated white blood cell count and ESR, but these can accompany inflammation of any cause and is not specific. In most patients extensive investigations have been performed to look for an infection before the diagnosis of Sweet's syndrome is made. Blood cultures and other tests for infection are negative.

Treatment and Outcome Sweets's syndrome does not respond to antibiotics. Because an infection is usually the first diagnosis suspected, most patients with Sweet's syndrome usually receive several courses of antibiotics. A corticosteroid such as prednisone 1 mg/kg a day is effective and rapidly reduces symptoms. The dose of corticosteroid is tapered over about six weeks. NSAIDs can improve joint symptoms and fever. sweets syndrome usually resolves completely but can relapse. Because Sweet's syndrome in adults can be the first sign of an underlying malignancy, a thorough physical examination and basic screening tests for malignancy should be performed.

synovectomy The synovium is normally a thin layer of tissue surrounding most of the joints of the body and lies inside the tough joint capsule. In addition, synovial tissue lines tendons where they are subject to friction and is also found within bursas. Since cartilage has no blood supply of its own, the synovium secretes nutrients into the joint space for the cartilage. It also contains nerves that feed back information about the state of the joint. In many forms of inflammatory arthritis (see arthritis for definition) the synovium enlarges, becoming packed with white cells that promote inflammation. New blood vessels form and fluid leaks into the joint, carrying enzymes and cytokines that cause damage to the cartilage as well as promoting more blood vessel growth and causing pain and swelling. To a certain degree this also happens in osteoarthritis, although much less aggressively.

Because the white cells and all the proinflam-matory molecules that they secrete enter by the synovial blood vessels, it seems logical that if the synovium is removed the arthritis will get better. Indeed this does happen, although it is not quite as simple as it once seemed. First the synovium will regrow, and second, removal of the synovium does not stop the cartilage from deteriorating. It may, however, dramatically relieve pain and swelling and improve function for months or years. There are two forms of synovectomy, surgical and radio-chemical.

Surgical This involves opening the affected joint and stripping out as much synovium as possible. More and more this is being done by arthroscopy. Although this is a less invasive procedure, doing a complete synovectomy arthroscop-ically is difficult. Most synovectomies are done in patients with rheumatoid arthritis. It is particularly indicated when one or two large joints are not responding to adequate medical therapy. There is sometimes a lot of synovitis around the tendons since they cross the back of the wrist, and this can lead to damage and rupture of the tendons. A synovectomy here is valuable in preventing rupture. synovectomy can sometimes relieve pain for up to 10 years, and about 70 percent of patients will have some benefit.

Patients with juvenile arthritis of the pauciar-ticular type often have one or two troublesome joints. If these can be successfully treated with syn-ovectomy, it may obviate the need to take potentially toxic medications. One study showed good pain relief in 70 percent of pauciarticular patients treated by synovectomy after seven years. In hemophilia, the large, swollen synovium may be a source of recurrent bleeding, and synovectomy can reduce the frequency of bleeds. However, synovec-tomy seems to cause a significant loss of movement in these patients. Surgical synovectomy is also the treatment of choice for a number of synovial diseases such as pigmented villonodular synovitis, synovial chondromatosis (see knee pain), and rare synovial tumors.

Radiochemical This involves injecting a radioactive substance into the joint to damage the synovium by irradiation. The substance needs to be relatively safe, easy to handle, and remain in the joint. Although a number have been used over the years, yttrium 90 is now used by most centers. Because it is a radioactive treatment, the center needs to be licensed to do it. A few centers commonly undertake this treatment for a wide referral area. Large joints such as knees, hips, and ankles are most commonly treated in this way. Smaller joints in the hands were successfully treated in the past, but radioactive injection was shown to be no better than corticosteroid injections in these joints and is therefore no longer done.

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