ADULT-ONSET STILL'S DISEASE

Photographs very kindly provided courtesy of Joseph M. Dyer, DO
Adult-onset Still's disease


The rash of AOSD is very similar to the rash of Still's disease but occurs in adults. A seronegative arthritis is associated and usually affects the wrists, ankles and knees. Pain is often most prominent with the rash. Onset of this disease is usually in the 20's.

Diagnostic criteria (need the presence 5 with at least 2 being major)

Major criteria

Minor criteria

Clinical

The rash is evanescent, often nocturnal and correlates with a temperature spike. The lesions are urticarial, pink or Salmon-colored and typically occur on the trunk and proximal extremities.

Ferritin

Measuring the ferritin can be very helpful in establishing the diagnosis of AOSD [J CLin Aesth Dermatol 2015;8(11;53-55)]. Very high levels, e.g., up to 75,000 ng/mL may be found in AOSD. Levels of ferritin above 1000 ng/mL are suggestive of AOSD and levels above 4000 ng/mL are very specific.

Treatment

A rheumatologist should be involved in diagnosis and treatment. A typical approach for the rash is antihistamines (may or may not help) and topical steroid (e.g. triamcinolone). The arthritis may be treated with non-steroidal anti-inflammatory drugs. Prednisone may be needed in severe cases. Other treatments for AOSD include methotrexate, cyclosporin, hydroxychloroquine, gold, penicillamine and azathioprine.

Associations with malignancy have been reported [JAAD 2015;73;294], but the exact link if any has yet to be fully elucidated.

Additional Pictures

Photographs very kindly provided courtesy of Joseph M. Dyer, DO Adult-onset Still's disease
Adult-onset Still's disease Adult-onset Still's disease

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