Erythema elevatum diutinum

Erythema elevatum diutinum (EED) is a disorder of the skin associated with small blood vessel inflammation (vasculitis). EED symptoms begin in adulthood and can last for many years. Many cases get better on their own, but this may take many years.

Epidemiology

EED is rare, with descriptions of only several hundred cases in the literature. The disease can develop at any age but is more common in the fourth and sixth decades. An earlier onset occurs more often in the setting of HIV infection. The male-to-female ratio is approximately equal, and no racial predilection has been observed.

Cause

The cause of EED is not well understood, but it is thought to be secondary to immune complex deposition in dermal blood vessels, which results in complement fixation and subsequent inflammation. The findings appreciated on examination of the skin may be seen due to deposition of immune complexes in small vessels, which leads to activation of complement, the influx of neutrophils, and the emission of destructive enzymes. Antineutrophil cytoplasmic antibodies may be pathogenic in EED. Furthermore, the initiation of EED may occur via activation of cytokines (interleukin-8), which causes selective recruitment of leukocytes to blood vessels. This leads to repetitive damage to the vessels and ultimately develops into fibrosis.

Symptoms

Symptoms include red, purple, brown or yellow bumps of different sizes that grow on or just below the skin. These growths are located mainly on the elbows, knees, ankles, hands, and fingers. People with EED may also have joint pain, but few other symptoms. The following list includes the most common signs and symptoms in people with erythema elevatum diutinum (EED). These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition. Symptoms may include:

• Yellow, purple, red and brown bumps on the skin (papules, nodules)
• Red, itchy patches of skin (plaques)
• Pain or burning
• Joint pain (arthralgia)

Symptoms usually begin between ages 40 and 60. Skin changes most often start on the knees, elbows, hands or fingers, and then move to the trunk. The skin changes may increase in size and number over many years. EED can last for several decades and then symptoms start to improve. Recovery is slow and may take 5 to 10 years.

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Arthralgia(Joint pain)
• Skin rash
• Vasculitis in the skin

30%-79% of people have these symptoms

• Abnormal blistering of the skin(Blistering, generalized)
• Increased circulating antibody level
• Skin vesicle

5%-29% of people have these symptoms

• Myalgia(Muscle ache)
• Skin nodule

Diagnosis

A skin biopsy is the most useful study for the diagnosis of EED. Electron microscopy is not a necessary diagnostic tool but would show changes and features of leukocytoclastic vasculitis. The erythrocyte sedimentation rate in patients with EED is often elevated. Antineutrophil cytoplasmic antibodies of IgA class may be helpful as a marker of disease, and Immunoelectrophoresis can also be used to identify possible gammopathies.

Treatment

Treatment of erythema elevatum diutinum is based on managing the symptoms. Medications such as anti-inflammatory drugs, antibiotics, and other drugs that fight infections. The skin growths may recur if medication is stopped. In some cases, surgery may be necessary to remove the skin growths. Dapsone, a sulfonamide antibiotic that impairs neutrophil chemotaxis and function, is the most effective treatment agent, but relapse is common upon discontinuation. It is also ineffective in nodular lesions, due to the fibrosis of the lesions. Other therapies include NSAIDs, niacinamide, tetracyclines, chloroquine, colchicine, and plasmapheresis. Topical and intralesional corticosteroids may be helpful for mild cases, but systemic corticosteroids are rarely indicated Antimicrobials, such as tetracyclines, are thought to alter neutrophil chemotaxis and phagocytosis, suppressing neutrophil chemotaxis and random migration in vivo. Niacinamide, an in vitro specific inhibitor of cyclic AMP, acts as a transfer factor needed for the suppression of antigen-induced lymphocyte transformation. It is relatively non-toxic and can be used in the treatment of neutrophil-driven disorders, with fewer side effects than dapsone. Local surgical excision can be beneficial for localized fibrotic nodules of EED. Regardless of treatment, the recurrence rate of EED is high if the underlying triggering factors are not controlled.

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NIH genetic and rare disease info

• NIH info on Erythema elevatum diutinum

Erythema elevatum diutinum is a rare disease.

Erythema elevatum diutinum Resources
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