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Erythema Elevatum Diutinum (EED)

Posted by: Cleo Libonati, RN, BSN in A LISTING OF ALL HEALTH CONDITIONS, Skin Disorders, Symptoms, Atypical, System - Integumentary (body surfaces) 0 5,866 Views

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erythema elevatum diutinum of forearmWhat Is Erythema Elevatum Diutinum?

Erythema elevatum diutinum is a rare leucocytoclastic vasculitis thought to be caused by an immune response that is characterized by inflammation, a rash, and scarring of small blood vessels of the skin.

A characteristic poorly defined rash develops symmetically in the skin of extremities where the vessels are inflamed. Nodules and plaques are yellowish red and crust on healing, leaving darkened areas (hyperpigmentation).

Q: What is leucocytoclastic vasculitis?

A: Leucocytoclastic vasculitis is the most common form of vasculitis (inflammation of blood vessels) of the skin and usually results from deposition of immune complexes at the vessel wall. It presents in different forms and in association with different diseases. Diagnosis is made by skin biopsy with immunofluorescence.1

Erythema elevatum diutinum may be caused by inflammatory disease, certain infections such as HIV, immunological disease, malignancy, and drugs.

Corticosteroids are indicated when there are signs of incipient skin necrosis or when the disease is severe. Otherwise, treatment is with Colchicine or Dapsone medications.2

What Is Erythema Elevatum Diutinum In Celiac Disease and/or Gluten Sensitivity?

  • Erythema elevatum diutinum is an immune vascular disorder associated with celiac disease.3
  • In celiac disease, the vasculitis is caused by deposition of antigen-antibody immune complexes, or other immune-mediated events.
  • The association between celiac disease and other immune disorders may be due to the sharing of a common genetic background, such as HLA antigens. However, in a very large study, involving 909 patients with celiac disease, Ventura and his associates found that the development of immune disorders in celiac disease was clearly related to the duration of exposure to gluten.4
  • Treatment with Dapsone is ineffective for clearing erythema elevatum diutinum in celiac disease. However, the lesions disappeared after a strict gluten-free diet was begun as described in cases reports below.5,6,7

How Prevalent Is Erythema Elevatum Diutinum In Celiac Disease and/or Gluten Sensitivity?

Erythema elevatum diutinum is documented in association with celiac disease.3

What Are The Symptoms Of Erythema Elevatum Diutinum?

  • Erythema elevatum diutinum is marked by red, purple, brown, or yellow papules, plaques, or nodules in the skin that crust when healing and leave a darkened area when healed.

How Does Erythema Elevatum Diutinum Develop In Celiac Disease and/or Gluten Sensitivity?

  • Erythema elevatum diutinum results from immune mechanism involving gluten exposure that damages small blood vessels in the skin.

Does Erythema Elevatum Diutinum Respond To Gluten-Free Diet?

Yes. Celiac disease-related sores disappeared on a strict gluten free diet in reported cases.3,8,7

6 Steps To Improve Erythema Elevatum Diutinum In Celiac Disease and/or Gluten Sensitivity:

Treatment. This condition responds to the complete elimination of gluten, which is the required treatment that improves both erythema elevatum diutinum and gut health.

  • Gut health is the foundation to restore ALL health. Restored health will enable you to maintain a strict gluten free diet, just as other life tasks will be easier.
  • A strict gluten free diet means removing 100% of wheat, barley, rye and oats from the diet.
  • Cutting out bread and other obvious sources of gluten is not good enough for recovery. Even 1/8th teaspoon of flour or bread crumb is enough to sustain the inflammation that is damaging your small intestine, causing increased permeability (leaky gut) and allowing undigested gluten to enter your body where it can damage structures and function, and instigate immune inflammatory responses.

Correct Your Individual Nutritional Needs.

Recovery. You should begin to feel better within a week and notice more energy as inflammation subsides and the  absorbing cells that make up the surface lining of your small intestine are better able to function.

  • Intestinal lining cells are replaced every 5 days. The healing process is like sunburn where the damaged surface layer of skin sloughs off and is replaced with new normal cells.
  • Leaky gut normally resolves in two month after starting a gluten free diet and brings about a big improvement in health. Improvement in intestinal permeability precedes morphometric recovery (cell appearance and structure) of the small intestine in celiac disease.9
  • The intestinal lining may take up to a year to heal.
  • 2 Reduce Inflammation. Foods to Eat and Foods Not to Eat:

Because gluten is inflammatory, eliminate OTHER inflammatory foods from your diet to reduce an additive effect to gluten. At the same time, try to eat foods that reduce inflammation (anti-inflammatory).

Here Are Major Inflammatory Food Types That Reduce Healing:

  • Damaging Foods. In susceptible persons, includes corn, dairy (cow), and soy. Lactose, the sugar in any animal milk disrupts intestinal permeability causing leaky gut.10
  • Allergenic Foods. Includes foods that trigger the immune sytem to produce IgE antibodies. Allergy testing is the usual way to discover these offending foods.
  • Shelf Stable Processed Foods. Includes any that contain additives and preservatives. Look for them on the nutrition label of the box or package. Additives and preservatives also disrupt intestinal permeability causing leaky gut.10
  • Fats. Limit deep fried foods, trans-fats, saturated fats (animal fat/butter), and EXCESSIVE omega-6 fatty acid oils like corn oil. Rancid fats, sodium caprate (a medium chain fat), and sucrose monester fatty acid (a food grade surfactant) induce significant disruption of the intestinal barrier that causes leaky gut.10.
  • Excessive Refined White Flours (bran layer removed). Includes products made from them such as cookies, bread, cakes, pies. Bran contains the vitamins and minerals that metabolize grains and slows the otherwise rapid entry of sugar from their digestion into the bloodstream. Also disrupt intestinal permeability causing leaky gut.10
  • Refined Sugars.  Includes white sugar, corn fructose and high fructose corn syrup.
  • Certain Spices. Includes paprika and cayenne pepper which disrupt intestinal permeability causing leaky gut.10
  • Alcohol and Caffeine. Disrupt intestinal permeability causing leaky gut.10

Here Are Important Anti-Inflammatory Food Types to Promote Health:

  • Fruits. Contain ample amounts of vitamins, minerals and phytochemicals which are naturally occuring components in plants that detoxify toxins, carcinogens (reducing the risk by 50%) and mutagens.
  • Non-Starchy Vegetables. Support intestinal integrity and provide ample amounts of vitamins, minerals and phytochemicals. Includes green leafy vegetables such as lettuce and kale, also onion, broccoli, garlic, and others.
  • High Quality Complex Carbohydrates. Provide vitamins, minerals, and fiber while boosting serotonin levels to help you relax and feel calm. Includes whole grains, legumes, and root vegetables such as carrots, parsnips, sweet potatoes, turnips, red beets, and others.
  • Antioxidants. Protect the body from inflammatory oxidant molecules that continually occur and help us handle stress and reduce irritability. Includes vitamin C-containing foods such as lemon, grapefruit, apricot, Brussels sprouts and strawberries, and others. Also, includes vitamin E-containing foods such as nuts, seeds, avocado, olive oil, and others. Cocoa is good, too.
  • Omega-3 Fatty Acids. Balance opposing omega-6 fatty acids and bad fats. Fish sources includes tuna, salmon, cod, and others. Plants sources include flax, chia seeds, canola oil, and others.
  • Probiotics. Supply normal microbes needed for colon health and health of the body such as these fermented foods: yogurt, kefir, and unpasteurized apple cider vinegar.
  • Prebiotics/ High Fiber Foods.  Food with fiber keeps our population of colonic microbes healthy.
  • Protective Herbs and Spices.  See below #6 below for examples.
  • 3 Information Sheet You Can Take to Your Doctor or Other Health Professional:

Click here.

  • 4 Manage Your Medications Safely:

Certain medications used to treat erythema elevatum diutinum deplete nutrients especially with long term use. Ask your doctor or pharmacist about this possible adverse effect if you are taking any of the drugs listed below. Do not stop prescribed medications without supervision.

 This is not a complete listing.

ANTIBIOTICS disrupt intestinal permeability which complicates celiac disease.

ANTI-INFLAMMATORIES disrupt intestinal permeability which complicates celiac disease.

  • 5Nutritional Supplements To Help Correct Deficiencies:

The type and quantity of nutritional supplements that may be needed depend on which nutrients are deficient.

  • Multivitamin/mineral combination that provides 100% once a day is useful to improve overall nutrient levels. This is a safe dose, but always check with your doctor to avoid interactions with medications.
  • B-Complex vitamins as prescribed if using steroids.
  • Calcium citrate as prescribed if using steroids. Calcium carbonate is pooly absorbed.
  • Ferrous fumarate or gluconates as prescribed if using steroids following a blood test for iron status.
  • Chelated magnesium  as prescribed but do not take at same time as calcium because they compete for absorption.
  • Vitamin K as prescribed if using Dapsone following a blood test for K status.
  • Zinc, Chromium or selenium as prescribed.

Storage Note: Store container tightly sealed, away from heat, moisture and direct light to avoid loss of potency. That is, in a safe kitchen cabinet – not in the bathroom or on the kitchen table.

  • 6Manage Natural Remedies: 

Hydration:

  • Eight glasses of water are recommended per day unless there is a contraindication such as kidney or heart disease. The Institute of Medicine recommends approximately 2.7 liters (91 ounces) of total water, from all beverages and foods, each day for women and 3.7 liters (125 ounces) daily of total water for men.
  • If you are thirsty, drink water. Add fresh, squeezed lemon to water. Lemon is anti-inflammatory, alkalizing and provides vitamin C.
  • Hydration Test: Urine should be pale yellow. Fingertips should be plump, without pruning but this may not be reliable when fingers are swollen with edema. Lips should be plump, without puckering. The feeling of thirst can be unreliable.
  • What is wrong with soda, coffee, tea, and alcohol? These drinks are dehydrating, increase acid, and deplete nutrients.

Carminatives. The following  anti-inflammatory plant sources called carminitives help heal the digestive tract. They also tone the digestive muscles which improves peristalsis, thus aiding in the expulsion of gas from the stomach and intestine to relieve digestive colic and gastric discomfort.

Carminative Food Remedies:

  • Raspberry.
  • Carrot is also a cleansing digestive tonic.
  • Grape is also bile stimulating and a cleansing remedy for sluggish digestion and laxative.
  • Redbeets also stimulate and improve digestion and are easily digested.
  • Cabbage also stimulates and improves digestion and is also a liver decongestant.
  • Lettuce also stimulates and improves digestion and is also an alterative, meaning it improves the function of organs involved with the digestion and excretion of waste products to bring about a gradual change.
  • Potatoes are antispasmodic (due to atropine like properties) and a liver remedy.

Carminative Herb Remedies:

  • Sage is also a digestive, astringent, bile stimulant and energy tonic that heals the mucosa.  Drink as tea or use in cooking.
  • Chamomile, lemon balm, and fennel, (as a tea) also help relieve nervous tension.
  • Parsley also relieves indigestion.
  • Rosemary as a tea and in cooking also is a nervous system tonic for stress and fatigue, bile stimulant, and can relieve headaches and indigestion.
  • Thyme is also soothing remedy useful for stimulating digestion of rich, fatty foods.

Carminative Spice Remedies:

  • Cloves are also antispasmodic.
  • Nutmeg is also useful for indigestion.
  • Ginger.

Exercise Helps:

Exercise improves circulation and rids the body of toxins.

Note: Exercise is important, but the amount and type of exercise undertaken depends on your health. Your first priority is to heal.

What Do Medical Research Studies Tell About Erythema Elevatum Diutinum In Celiac Disease and/or Gluten Sensitivity?

CASE REPORT SUMMARIES

“Macroamylasemia as the first manifestation of celiac disease.” This case report describes diagnosis of celiac disease in a 52-year-old woman who was referred to an outpatient clinic with a 6-month history of weakness, weight loss of 6 kg, maculopapular rash on her legs and persistent hyperamylasemia for 3 months (amylase >1,400 U [normal 35–120 U] and very low 24-hour urine amylase and amylase clearance/creatinine clearance ratio (1.9% [normal 3.0 ± 1.1%]), consistent with macroamylasemia.

Her medical history was remarkable only for several years of chronic normocytic normochromic anemia (Hb 9.9 g%) and osteoporosis. She was not receiving any medications. Other than a sallow pallor and the maculopapular rash on both legs, the physical examination was completely negative. Laboratory results: thrombocytosis (510,000 platelets/ml), albumin 3.1 g, AST 60 U (normal 15–30), rheumatoid factor 81 (normal 0–15), anti-mitochondrial antibody (AMA) 1:80. The rest of the laboratory results were normal. An abdominal computerized tomogram with contrast yielded no pathological findings.

Biopsy from the skin lesions on her legs revealed leukocytoclastic vasculitis (erythema elevatum diutinum).

The patient had macroamylasemia and anemia, which can present as an autoimmune disease, such as celiac disease. Her serology for celiac disease was strongly positive, and she underwent esophagogastroduodenoscopy which revealed scalloping of folds in the second part of the duodenum. A histological evaluation confirmed the diagnosis of celiac disease. The patient was started on a gluten-free diet which led to a significant clinical improvement two months later: she felt generally stronger, had gained weight, the rash had disappeared, the serum amylase values were normal, hemoglobin had increased to 11.7 g%, and there was a return of serum albumin to normal values.7

“Erythema elevatum diutinum in association with coeliac disease.” This case report of a female presenting with EED describes subsequent diagnosis of celiac disease based on duodenal biopsy revealing total villous atrophy. Appearance of EED lesions was preceded by widespread joint pain. In lesional skin, granular deposits of IgA and C3 were seen at the dermo-epidermal junction. Dapsone was partly effective, but complete healing of the EED lesions was achieved only after the introduction of a strict gluten free diet.11

“Erythema elevatum diutinum associated with celiac disease: response to a gluten-free diet.” This case report describes the course of an 11-year-old girl with a history of insulin-dependent diabetes mellitus who had erythema elevatum diutinum (EED) associated with a celiac disease related to a possible kidney disease. Dapsone did not improve the skin manifestations. However, the lesions disappeared after a gluten-free diet was begun. To our knowledge, this report describes the first case of EED in a patient with celiac disease.5

  1. Sunderkötter C, Bonsmann G, Sindrilaru A, Luger T. Management of leukocytoclastic vasculitis. J Dermatolog Treat. 2005;16(4):193-206. [↩]
  2. Sunderkötter C, Bonsmann G, Sindrilaru A, Luger T. Management of leukocytoclastic vasculitis. J Dermatolog Treat. 2005;16(4):193-206. [↩]
  3. Tasanen K, Raudasoja R, Kallioinen M, Ranki A. Erythema elevatum diutinum in association with coeliac disease. The British Journal of Dermatology. Apr 1997;136(4)624-7. [↩] [↩] [↩]
  4. La Villa G, Pantaleo P, Tarquini R, Cirami L, Perfetto F, Mancuso F, Laffi G. Multiple immune disorders in unrecognized celiac disease: a case report. World J Gastroenterol. 2003;9(6):1377-1380, Available at: http://www.wjgnet.com/1007-9327/9/1377.asp. Accessed Jan 3, 2005. [↩]
  5. Rodriguez-Serna M, Fortea JM, Perez A, Febrer I, Ribes C, Aliaga A. Erythema elevatum diutinum associated with celiac disease: response to a gluten-free diet. Pediatr Dermatol. 1993 Jun;10(2):125-8. [↩] [↩]
  6. Tasanen K, Raudasoja R, Kallioinen M, Ranki A. Erythema elevatum diutinum in association with coeliac disease. The British Journal of Dermatology. Apr 1997;136(4)624-7. [↩]
  7. Depsames R, Fireman Z, Niv E, Kopelman Y. Macroamylasemia as the first manifestation of celiac disease. Case Rep Gastroenterol. 2008 Jun 6;2(2):196-8. doi: 10.1159/000132771. [↩] [↩] [↩]
  8. Rodriguez-Serna M, Fortea JM, Perez A, Febrer I, Ribes C, Aliaga A. Erythema elevatum diutinum associated with celiac disease: response to a gluten-free diet. Pediatr Dermatol. 1993 Jun;10(2):125-8. [↩]
  9. Cummins AG, Thompson FM, Butler RN, et al. Improvement in intestinal permeability precedes morphometric recovery of the small intestine in coeliac disease. Clinical Science. Apr 2001;100(4):379-86. [↩]
  10. Farhadi A, Banan A, Fields J, Keshavarzian A. Intestinal barrier: an interface between health and disease. Journal of Gastroenterology and Hepatology. 2003;18:479-91. [↩] [↩] [↩] [↩] [↩] [↩]
  11. Tasanen K, Raudasoja R, Kallioinen M, Ranki A. Erythema elevatum diutinum in association with coeliac disease. The British Journal of Dermatology. Apr 1997;136(4)624-7. [↩]

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