Erythema elevatum diutinum

What is erythema elevatum diutinum?

Erythema elevatum diutinum (EED) is a rare type of necrotising vasculitis that is characterised by red, purple, brown or yellow papules (raised spot), plaques, or nodules, found on the backs of the hands, other extensor surfaces overlying joints, and on the buttocks.

Erythema elevatum diutinum

Erythema elevatum diutinum

Erythema elevatum diutinum

Who gets erythema elevatum diutinum?

EED may occur in any age group, but patients are typically between 30 and 60 years old. It occurs equally in men and women.

What is the cause of erythema elevatum diutinum?

EED is classified as a small vessel vasculitis. The cause of EED is not yet defined, but it has been associated with the following conditions:

• Granuloma faciale
• Recurrent bacterial infections (especially streptococci)
• Viral infections (including hepatitis B and HIV)
• Haematological diseases
• Rheumatological diseases

What are the clinical features of erythema elevatum diutinum?

• Lesions usually start as papules or nodules on the backs of the hands.
• Other extensor surfaces affected include the knees, elbows, wrists, ankles, fingers and toes. Buttocks, trunk, forearms, legs, palms and soles may also be affected.
• Lesions occurring on the face are indistinguishable from granuloma faciale.
• Lesions usually appear symmetrically.
• Colour of lesions progresses over time from yellow or pinkish to red, purple or brown.
• Lesions may enlarge during the day and go back to original size overnight.
• Rarely, blisters and ulcers may form.
• Lesions usually feel firm and freely movable over the underlying tissue.
• EED can be symptomless or painful or cause an itching or burning sensation.
• Symptoms can worsen after exposure to cold.
• Arthralgia may be present.

How is erythema elevatum diutinum diagnosed?

Skin biopsy is the most important test to establish a diagnosis of EED; it shows leukocytoclastic vasculitis. See Erythema elevatum diutinum pathology.

Direct immunofluorescence is useful to confirm the presence of an immune complex type of vasculitis.

What is the treatment for erythema elevatum diutinum?

EED is a chronic and progressive skin disease that may last as long as 25 years. However, in some cases after evolving over a 5–10 year period it may spontaneously clear.

Medication can be used to limit the progression of the disease.

Dapsone is considered the drug of choice for EED, mainly because of its rapid onset of action and clinical experience has shown good responses. However, lesions promptly recur following the withdrawal of the drug.

Other drugs that have been occasionally reported to be effective include:

• Niacinamide
• Colchicine
• Hydroxychloroquine
• Clofazimine
• Cyclophosphamide.

Oral corticosteroids are generally ineffective.

What is the outcome for erythema elevatum diutinum?

EED generally persists for months, years or decades. It often recurs after apparently successful treatment.