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Erythema Elevatum Diutinum

Last updated Sept. 26, 2018

Approved by: Krish Tangella MD, MBA, FCAP

Erythema Elevatum Diutinum (EED) is an uncommon condition that manifests as skin lesions usually on the arms and legs in middle-aged adults.

What are the other Names for this Condition? (Also known as/Synonyms)

  • EED (Erythema Elevatum Diutinum)

What is Erythema Elevatum Diutinum? (Definition/Background Information)

  • Erythema Elevatum Diutinum (EED) is an uncommon condition that manifests as skin lesions usually on the arms and legs in middle-aged adults
  • The cause of Erythema Elevatum Diutinum is not well-established. It is often associated with many autoimmune diseases, malignancies, blood-related conditions, and cutaneous disorders
  • Erythema Elevatum Diutinum is a chronic condition that progresses over time. The condition does not respond well to therapy, which is usually instituted to stop its progression and bring relief from the symptoms
  • A diagnosis of Erythema Elevatum Diutinum involves assessment of the clinical symptoms and skin/tissue biopsy. Following its diagnosis, the skin disorder is treated using medications and supportive measures
  • The treatment may involve dapsone therapy in many cases. Other measures may include therapeutic plasma exchange and surgery for severe skin conditions. The prognosis of Erythema Elevatum Diutinum is generally good, but the condition is difficult to cure and may last for many years

Who gets Erythema Elevatum Diutinum? (Age and Sex Distribution)

  • Erythema Elevatum Diutinum is observed in adults in the 30-60 years’ age group
  • Both males and females may be equally affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Erythema Elevatum Diutinum? (Predisposing Factors)

Currently, no risk factors have been clearly identified for Erythema Elevatum Diutinum. However, the condition is associated with an assortment of medical conditions that include:

  • Autoimmune disorders that include rheumatoid arthritis, celiac disease, inflammatory bowel disease, and diabetes mellitus
  • Bacterial infections including syphilis, streptococcal infection, etc.
  • Cancers such as multiple myeloma, B-cell lymphoma, breast carcinoma, myelodysplastic syndromes, etc.
  • Hypergammaglobulinemia
  • IgA monoclonal gammopathy
  • Relapsing polychondritis
  • Skin conditions such as pyoderma gangrenosum and granuloma faciale
  • Viral infections including hepatitis, HIV infection

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Erythema Elevatum Diutinum? (Etiology)

  • The exact cause of Erythema Elevatum Diutinum (EED) is currently unknown. This skin condition is associated with a variety of diseases and disorders
  • It is considered to be a low-grade form of leukocytoclastic vasculitis. Vasculitis is a condition in which inflammation of the blood vessels is noted; usually, the small blood vessels are affected
  • Research is being conducted to ascertain the underlying cause of EED

What are the Signs and Symptoms of Erythema Elevatum Diutinum?

The signs and symptoms of Erythema Elevatum Diutinum may include:

  • Presence of numerous skin lesions that may be in the form of papules, plaques, and nodules
  • The skin rashes may appear symmetrically on the body; any part of the body may be affected
  • The condition is sometimes associated with pain and itching of skin
  • Initially, the skin lesions are observed on the back of the hands. Later, it may involve the arms and legs (elbows, knees including the palms and soles), chest and back, and face
  • The skin over the lesions may be discolored yellow or red, which then changes to dark red, brown, or purple color
  • The soft and tender lesions may become firm, but freely-moving beneath the skin
  • In some individuals, the formation of fluid-filled blisters and skin blistering is noted
  • The skin condition can get worse during the day, or be aggravated on exposure to cold
  • Joint pain is noted in many cases, while muscle pain may be observed occasionally

Additionally, the signs and symptoms of any associated medical condition may be noted.

How is Erythema Elevatum Diutinum Diagnosed?

Erythema Elevatum Diutinum is diagnosed on the basis of the following information:

  • Complete physical examination and thorough medical history evaluation
  • Assessment of signs and symptoms
  • Laboratory tests that include blood test, which may show the presence of antibodies
  • Tests to diagnose associated health conditions, if any
  • Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
  • Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
  • Skin or tissue biopsy: A biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
  • Immunofluorescence studies on skin biopsy
  • In rare cases, electron microscopy may be performed
  • A differential diagnosis to exclude the following conditions may be necessary prior to a definitive diagnosis:
    • Sweet syndrome; which responds to steroidal therapy, while Erythema Elevatum Diutinum does not respond to systemic corticosteroids
    • Vasculitis involving small blood vessels

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Erythema Elevatum Diutinum?

The complications of Erythema Elevatum Diutinum may include:

  • Longstanding EED and cosmetic issues can result in severe emotional stress
  • Bleeding and ulceration of the skin may result in secondary viral or bacterial infections
  • Change in pigmentation of skin over time (hyperpigmentation or hypopigmentation)
  • Recurrence of the skin lesions on stoppage of medications
  • Complications that arise from associated conditions, if any present

How is Erythema Elevatum Diutinum Treated?

It is generally difficult to treat Erythema Elevatum Diutinum (EED). The treatment also depends on the associated condition. It may involve the following measures:

  • Symptomatic and supportive therapy to arrest progression of the condition
  • The commonly used medication is the antibiotic drug dapsone; other medications include hydroxychloroquine, cyclophosphamide, niacinamide, etc.
  • Topical steroid creams and oral medications are not helpful in treating EED
  • Intermittent therapeutic plasma exchange (PLEXA)
  • In some individuals, surgery is indicated for severe skin conditions

Treatment of the associated condition, in some cases, is known to relieve the symptoms of Erythema Elevatum Diutinum.

How can Erythema Elevatum Diutinum be Prevented?

Currently, it is not possible to prevent Erythema Elevatum Diutinum, since the cause of the condition is unknown.

What is the Prognosis of Erythema Elevatum Diutinum? (Outcomes/Resolutions)

The prognosis of Erythema Elevatum Diutinum is dependent upon the associated disorder/condition, severity of the symptoms, and the complications it presents.

  • Even though it is a benign condition, Erythema Elevatum Diutinum can progress and persist for several years to even a decade (5-10 years), after which it may disappear
  • Appropriate treatment can help decrease its progression. In very rare cases, EED may not present any symptoms and may go unnoticed
  • Individuals with mild conditions have better prognosis than those with severe symptoms and complications

Additional and Relevant Useful Information for Erythema Elevatum Diutinum:

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Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Sept. 26, 2018
Last updated: Sept. 26, 2018