What is Erythema Elevatum Diutinum?
Erythema elevatum diutinum (EED) is a rare and long-lasting skin condition. It is marked by red to brown bumps, patches, and lumps, often found on the outer parts of the body like arms and legs. These skin changes usually don’t cause any symptoms, although some people might feel pain or a burning sensation. Aside from skin symptoms, some people with this condition might also experience joint pain, fever, or other general discomforts. While EED itself isn’t harmful, it can sometimes be linked to infections, blood disorders, immune system diseases, or other health conditions. Normally, these skin changes get better on their own after around 5 to 10 years.
What Causes Erythema Elevatum Diutinum?
Eosinophilic dermatosis of hematologic malignancy (EED) is a skin condition whose cause remains unclear. It’s believed to occur due to the buildup of immune complexes (proteins that are produced when your body fights off a disease) in the small blood vessels of the skin. This buildup triggers a response from the body’s immune system, leading to inflammation or swelling.
Typically, these immune complexes cause a chain reaction in the body. The initial response activates what’s known as ‘complement,’ part of our immune system that helps antibodies fight off harmful substances. This draws in a type of white blood cell called neutrophils, which release enzymes that can damage tissues.
Interestingly, it’s been suggested that certain types of antibodies (antineutrophil cytoplasmic antibodies) might play a role in causing EED. Additionally, EED’s onset might be linked to the activation of certain substances in the body, known as cytokines (specifically, interleukin-8). These cytokines attract more white blood cells to the blood vessels, causing repeated damage and leading to fibrosis (thickening or scarring) of the blood vessel tissue over time.
Risk Factors and Frequency for Erythema Elevatum Diutinum
EED, or Erythema Elevatum Diutinum, is a quite rare condition. There are only a few hundred documented cases so far. While it can occur at any age, it’s often seen in people in their 40s and 60s. However, it’s worth noting that it tends to occur earlier in those who have HIV. Men and women are affected equally and it doesn’t appear to affect any particular racial group more than others.
Signs and Symptoms of Erythema Elevatum Diutinum
EED, or Erythema Elevatum Diutinum, is a skin condition where you might see red-brown, yellowish, or purple bumps, patches, or lumps on your skin. These symptoms tend to appear in a symmetrical pattern and are more likely to be found around joints and on the limbs – particularly the outer parts of the elbows, knees, ankles, hands, and fingers. Other places you might see these skin changes include the face, behind the ears, underarms, buttocks, and private parts. It’s not common to see them on the trunk of the body. The affected skin areas are usually firm and move freely over the tissue underneath, except if they’re on the palms of the hands or soles of the feet.
When these skin areas first appear, they are usually red and soft. But over time, they become red-brown or purple, and feel firm to touch due to fibrosis or tissue thickening. These skin changes can vary in color, size, and number throughout the progression of the disease.
- Red-brown, yellowish, or violaceous bumps, patches, or lumps
- Skin changes apparent around joints and on the limbs
- Changes in face, behind ears, underarms, buttocks, or private parts
- Firmness and mobility of changes over underlying tissue
Normally these skin changes don’t cause pain or discomfort, but some individuals have reported itchiness and burning or tingling sensations, especially in the early stages of the disease. In people living with HIV, EED often presents as larger lumps that can grow into hefty masses. Pain might be felt in the joints under the skin changes, and there have been rare reports of eye irregularities such as inflammation of the outer layer or interior of the eye and degeneration of the cornea. However, other systems in the body are hardly ever affected.
EED is a chronic condition, which means it’s long-lasting, and its course can come and go. Most cases gradually get better on their own over a period of 5 to 10 years, but it can sometimes last up to 40 years. EED skin changes usually don’t leave scars but can leave areas of darker or lighter skin behind. Nobody has ever died from EED.
- Itchiness, burning or tingling sensations
- Larger lumps in people with HIV
- Pain in joints underneath skin changes
- Rare cases of eye irregularities
- Long-lasting condition with a come-and-go course
- Changes usually resolve on their own over 5 to 10 years
- Darker or lighter areas of skin left behind
- No reported fatalities due to EED
Testing for Erythema Elevatum Diutinum
A skin biopsy is often the most helpful tool for diagnosing EED, a skin disease. It’s not necessary, but under an electron microscope, the skin changes would appear similar to leukocytoclastic vasculitis, a form of blood vessel inflammation. Another test that could indicate EED is called the erythrocyte sedimentation rate, which can be typically higher in people with this condition.
Furthermore, a type of protein called Antineutrophil cytoplasmic antibodies from the group IgA may be present and serve as a marker of the disease. Immunoelectrophoresis is another test which can help identify any abnormal proteins in the body, specifically ones typically associated with disorders of the immune system.
Treatment Options for Erythema Elevatum Diutinum
Treating EED, a chronic condition, can be quite challenging. EED often reappears from time to time, making it difficult to manage.
Dapsone, an antibiotic, is most effective for treating EED. However, stopping the medication can lead to a relapse of the illness. Furthermore, dapsone is not effective on nodular lesions which are hardened patches due to scar tissue. Other treatment options for EED include Non-Steroidal Anti-Inflammatory Drugs (NSAIDs), niacinamide, certain antibiotics (like tetracyclines), chloroquine, colchicine, and plasmapheresis, a procedure where the plasma in your blood is filtered or changed.
For milder cases of EED, corticosteroids that you apply directly to your skin or injected directly into lesions can be helpful. However, corticosteroids that are taken orally are rarely recommended.
Antibiotics like tetracyclines can help to modify the movement and functioning of a type of white blood cell called neutrophils. Niacinamide, a form of Vitamin B3, is also helpful in treating conditions driven by neutrophils, like EED, and is generally less toxic with fewer side effects than dapsone. For localized, hardened patches of EED, surgical removal can be beneficial.
Regardless of the treatment used, it’s important to note that EED often comes back, particularly if the factors that trigger the condition are not managed well.
What else can Erythema Elevatum Diutinum be?
When it comes to skin conditions, the following diseases may exhibit similar symptoms, making them potential considerations for doctors:
- Acute febrile neutrophilic dermatosis
- Dermatofibroma
- Erythema multiforme
- Granuloma annulare
- Granuloma faciale
- Multicentric reticulohistiocytosis
- Pyoderma gangrenosum
- Xanthomas
