Celiac.com 04/25/2012 - In my experience growing up with undiagnosed celiac disease, I had to deal with several symptoms that my doctors had no answers for. One of the most frustrating of these was my skin troubles—dermatitis herpetiformis. After my experiences with misdiagnoses, and finally more recently, learning how to effectively get rid of dermatitis herpetiformis, I encourage parents to be particularly watchful for signs of dermatitis herpetiformis in their children, and I have some useful advice for those—children and adults—who have already been diagnosed with this annoying and sometimes quite troublesome rash. Since dermatitis herpetiformis occurs in 15 to 20% of celiacs, it’s worth any celiac’s time to learn more about this condition.

By definition, dermatitis herpetiformis is a blistering and extremely itchy skin rash. It’s usually symmetrical in shape and is most commonly located on the elbows, knees, buttocks, and upper back. It’s common for people with dermatitis herpetiformis to have rashes appear in the same spot, and they can either be consistent or come and go. People can experience the rash on other parts of the body, and severity of symptoms can vary. Dermatitis herpetiformis is sometimes called the “gluten rash” or “celiac disease rash” because it occurs in people with a gluten intolerance or celiac disease. It is commonly misdiagnosed as eczema.

Gluten is a protein found in wheat, barley, and rye. In people who have celiac disease, gluten causes an autoimmune response which results in the immune system attacking the lining of the small intestine—specifically the villi, the absorptive hair-like structures of the lining. With dermatitis herpetiformis, outbreaks are also triggered by gluten.

Interestingly, unlike celiac disease which appears more in women than men, dermatitis herpetiformis is more commonly found in men by a ratio of about two-to-one. It is rarely seen in children under ten and first appears in the teenage years or even in one’s twenties or thirties. It may come and go, even if you’re eating a gluten-containing diet.

Diagnosis is done with a skin biopsy. In most cases, a dermatitis herpetiformis diagnosis means celiac disease as well, even if you’re not obviously suffering from the characteristic intestinal symptoms of this disease. No matter what, the treatment is the same: a strict gluten-free diet.

Dermatitis herpetiformis rashes are treated in two main ways--the gluten-free diet, of course, and antibiotics such as dapsone or sulfapyridine for those who aren’t able to tolerate dapsone. A truly gluten-free diet can eliminate dermatitis herpetiformis, but in my experience and according to the National Institutes of Health, a dermatitis herpetiformis rash responds dramatically to dapsone, within 48 to 72 hours. To treat the underlying cause of dermatitis herpetiformis, which is celiac disease, a strict gluten-free diet must be followed, but according to the National Institutes of Health, “Even with a gluten-free diet, dapsone or sulfapyridine therapy may need to be continued for 1–2 years to prevent further dermatitis herpetiformis outbreaks.”

As a celiac with dermatitis herpetiformis, completely eliminating gluten from my diet has been the only lasting solution for dermatitis herpetiformis, but unfortunately I can accidentally ingest gluten from time to time, especially when I travel. In my most recent outbreak, I decided to get a prescription for dapsone. Although dapsone is a very strong drug with side effects and should be used sparingly, I was in need of something fast-acting. I followed the instructions exactly, and not only did it relieve the pain but within three days, I could see a remarkable change in the appearance of the dermatitis herpetiformis. After reexperiencing the painful and frustrating symptoms of dermatitis herpetiformis and the relief that came with proper treatment, I knew I had to address this topic to help others. I encourage everyone to get the word out about dermatitis herpetiformis so more and more people dealing with this misdiagnosed condition can get help just as I did.

Resources:

• Open Original Shared Link.
• Open Original Shared Link.
• Open Original Shared Link.

Celiac.com 12/29/2011 - About one in 100 people in America has celiac disease, while about one in four of those will develop dermatitis herpetiformis Duhring, which occurs when celiac disease manifests cutaneously, in the skin. Dermatitis herpetiformis Duhring is uncommon in children, with only 5% of cases appearing in children younger than 7 years. Most often, it presents in people over forty.

Making a proper clinical diagnosis of dermatitis herpetiformis Duhring, also known as Duhring’s disease, is challenging, and often requires the help of skin biopsy and direct immunofluorescence.

To do this, clinicians should look for antibodies against gliadin, endomysium, and transglutaminase, said Dr. Magdalene A. Dohil, of the University of California, San Diego, at a seminar sponsored by Skin Disease Education Foundation (SDEF).

The fact that manifestations of celiac disease in the mucous and skin may point to Duhring's disease was one of the more important aspects of Dr. Dohil's discussion, for people with celiac disease, and those treating them.

Dr. Dohil noted that, at some point during the course of their disease, more than seven in ten people (74%) with celiac disease will have some type of skin manifestation. Most often, this skin manifestation occurs in the form of xerosis, which often triggers pruritus. Mucosal manifestations occur in 27% of patients, especially in patients with longer history of celiac disease.

Dr. Dohil pointed out numerous diseases, disorders, syndromes, and structural epithelial defects with clear connections between skin and gut. For example, 60%-82% people with asymptomatic inflammatory bowel disease present with mucocutaneous findings that include skin tags, fistulas, fissures, or abscesses in the perianal and genital areas. In 25%-30% of cases, these will precede GI complaints. Dr. Dohil said.

Overall, 6%-20% of all patients with inflammatory bowel disease develop oral lesions, but up to 80% of pediatric cases with Crohn’s disease and 41% with ulcerative colitis develop such lesions.

Source:

• Open Original Shared Link
  

Here are some common dermatological concerns associated with celiac disease:

• Dermatitits Herpetiformis—This painful, blistery condition can be very stressful, especially when misdiagnosed.  An inflamed, itchy rash, dermatitis herpetiformis begins as tiny white filled blisters or red spots around hair follicles.  Trying to hide or disguise DH, as well as trying to treat it when misdiagnosed can be incredibly stressful for a person.
• Eczema—Eating a gluten-free diet is becoming an increasingly popular mode of treatment for eczema.  Those who are gluten intolerant also tend to have more advanced psoriasis.Psoriasis—Like eczema, psoriasis has in many cases shown improvement when the person is put on a gluten free diet.  In Scott Adams’ 2004 article, he also mentioned that psoriasis in those with celiac tends to be more severe.
• Acne—Links between celiac and malabsorption, as well as hormonal upset can contribute to a greater production of acne.  Many birth control pills boast promises of clearer skin, their method is through hormone manipulation.  Because many who suffer from gluten intolerance also experience a disruption of normal hormone function, this disharmony can lead to problems with acne. 
• Dry Skin—Also correlated to malabsorption, dry skin is a very common complaint amongst those with celiac.  But this condition is one that many people see even after the prescribed treatment of a gluten free diet.  Why?  Vitamin E rich grains are vital to maintaining skin harmony, but since many who are gluten intolerant begin avoiding grains completely—even those grains that are gluten-free, getting that important Vitamin E in their diets can become a challenge.
  

In addition to being connected with a higher risk of celiac disease in both Dutch and Spanish populations, the gene has been associated with a higher risk of inflammatory bowel disease, systemic lupus, erythmatosus, and rheumatoid arthritis, which means that myosin IXB is likely a shared risk factor in all of these disorders.

Researchers looked at nearly 500 Hungarian and Finnish families, plus another 270 patients and controls. What they found was a substantial linkage to chromosome 19p13 (LOD 3.76 P=0.00002) that lends great weight to the notion that this is a substantial risk factor. Other variants of the myosin IXB gene showed no connection with celiac disease, though they did show a small connection to dermatitis herpetiformis.

Both phenotypes show a significant connection indicating that the role meaning that there still may be a role being played by nearby genes. They are calling for more comprehensive genetic and functional studies to determine what the exact nature of the role the myosin IXB gene in both celiac disease and in dermatitis herpetiformis.

As more studies are conducted, and more data emerges, we are likely to get a much clearer genetic picture of both celiac disease and dermatitis herpetiformis. A clearer genetic picture will likely lead to new and novel approaches to treatment that permit much more effective targeting of treatment.

Journal of Med. Genet. 2007 Dec 12

Gluten-free diets have been used in the treatment of patients with dermatitis herpetiformis in our department since 1967. Of the 212 patients with dermatitis herpetiformis attending between 1967 and 1992, 133 managed to take the diet, and 78 of these achieved complete control of their rash by diet alone. Of the remaining 55 patients taking a gluten-free diet, all but three were taking partial diets; over half of these patients managed to substantially reduce the dose of medication required. Of the 77 patients taking a normal diet, eight entered spontaneous remission, giving a remission rate of 10%; a further two patients who had been taking gluten-free diets were found to have remitted when they resumed normal diets. Loss of IgA from the skin was observed in 10 of 41 (24%) patients taking strict gluten-free diets. These patients had been taking their diets for an average of 13 years (range 5-24 years), and their rash had been controlled by diet alone for an average of 10 years (range 3-16 years). The advantages of a gluten-free diet in the management of patients with dermatitis herpetiformis are: (i) the need for medication is reduced or abolished; (ii) there is resolution of the enteropathy, and (iii) patients experience a feeling of well-being after commencing the diet. Thus, we propose that a gluten-free diet is the most appropriate treatment for patients with dermatitis herpetiformis.

The following report comes to us from The Sprue-Nik Press, which is published by the Tri-County Celiac Sprue Support Group, a chapter of CSA/USA, Inc. serving southeastern Michigan (Volume 7, Number 5 July/August 1998 Dermatitis Herpetiformis). Dr. Kim Alexander Papp is a consultant at St. Marys, Grand River, and Listowel Memorial Hospitals. He is also President of Probity Medical Research Inc.

The first mention of Dermatitis Herpetiformis (DH) in the literature was in 1884 in Dhring. The connection to wheat was made in Dreke, Holland in 1941. It is an uncommon, but not rare, disease that affects males twice as often as females. It is found in 10% of first degree relatives. There is a genetic association; 90% of DH patients have HLA-B8 vs. only 15% of the general population. HLA-DRw4 and HLA-DQw2 are also associated with some DH patients.

DH normally is found on elbows, knees, shoulders, buttocks, sacrum, posterior scalp, and face. While it is unusual, it can also show upon the hands or inside the mouth. It presents as clear blisters that itch very badly. [One patient described the itch ...like rolling in poison ivy naked with a severe sunburn, then wrapping yourself in a wool blanket filled with ants and fleas.-ed]

The original diagnosis of DH was done by giving Dapsone, a leprosy drug, and noting any improvement. Today, the gold standard for diagnosing DH is a skin biopsy with immunofluorescence. (A plain skin biopsy is not sufficient.) Most DH patients also have villi damage in the small intestine and lymphocyte infiltration of the intestinal wall, and IgA/IgG antigliadin antibodies in the bloodstream. However, there is really no need to perform a small bowel biopsy or test for blood serum antibodies; the skin biopsy with immunofluorescence provides a definitive diagnosis.

Dr. Papp indicated that about half of his patients are diagnosed after having their symptoms recognized and pointed out to them by other DH patients.

DH is not an allergic reaction; a different mechanism is involved. It is caused by antibodies to the gluten found in wheat, rye, and barley.

The causes of DH flares include large quantities of iodides (some iodine is needed in the diet), kelp, shellfish, non-steroidal anti-inflammatory agents (such as aspirin), gluten, stress, and some cleansers.

What else looks like DH?

• DH can be misdiagnosed as psoriasis, or the patient may have both conditions.
• Linear IgA disease--the immunofluorescence pattern is different, but it looks and feels the same as DH to the patient.
• Allergic contact reactions.

DH is treated by adherence to a gluten-free (gluten-free) diet. The skin lesions can be treated with either a sulfone (Dapsone) or sulfonamide(Sulfapyradine) drug. In about 85% of the cases, at least a year on a strict gluten-free diet is needed before DH is resolved. In rare cases DH lesions clear up after only a few weeks on the gluten-free diet.

Dapsone can have side effects, though these are not common. It can alter blood chemistry, causing anemia. Those of Mediterranean or African ancestry can have sudden red blood cell count drops [known asG6PD Deficiency--Dr. Alexander]. Other complications include tingling fingers and neurological problems.

Ideally, if the patient is on medication there would be monthly lab tests to monitor the dosage and effect on the patient. This almost never happens.

The gluten-free diet takes a long time to bring DH under control because it requires time to clear the IgA and IgG from the blood. So even if one is on a gluten-free diet and/or taking Dapsone, technically one has DH. Like an alcoholic, one always has the disease.

Dr. Papp concluded his presentation by answering a few questions from the audience:

Q: How soon after ingesting gluten or iodine will a flare occur?
A: It varies tremendously. With iodine, it usually takes several days of consumption before a flare occurs.
Q: What effect does stress have on a DH patient?
A: It intensifies any symptoms the patient is experiencing.
Q: What effect does iodine on the skin have?
A: It really has no effect; it doesnt penetrate enough. Iodine must be consumed to cause a DH flare.
Q: After several years on a gluten-free diet with no flares, is iodine still a problem?
A: No.

The following was written by Dr. Joseph Murray, one of the leading USA physicians in the diagnosis of celiac disease (celiac disease) and dermatitis herpetiformis (DH). Dr. Murray (Open Original Shared Link) of the Mayo Clinic Rochester, MN, is a gastroenterologist who specializes in treating Celiac disease:

In response to your questions about DH, The following represents my views about this curious and very itchy condition. In general DH is a severely itchy skin condition that often starts abruptly, affecting the elbows knees buttocks and scalp and the back. It usually starts as little bumps that can become tiny blisters and then are usually scratched off. It can occur in one spot only but usually occurs in many different areas. The condition is related to the deposit under the skin of IgA deposits. These occur in response to the ingestion of gluten in the diet. However, once deposited there, they are only slowly cleared by the body even when the individual is gluten free. While most individuals with DH do not have obvious GI symptoms almost all have some damage in their intestine. They the potential for all of the nutritional complications of celiac disease.

The diagnosis is made by taking a skin biopsy and performing immunoflorescence studies on it (a specialized type of stain in major laboratories) The test is usually reliable but it takes a significant dedication to detect early cases where there is a short history of rash rather than years or months of rash. It is unusual to develop DH after the start of a GFD for celiac disease. About 5 % of celiac disease patients will develop DH usually in the first 6-12 months. This probably reflects the long lasting nature of the deposits under the skin.

Treatment for DH is twofold. (1) Remove the cause: gluten. (2) Suppress the skin response with drugs such as Dapsone or some other sulphones. The latter is the most common treatment used as it is rapidly relieves the itch. However there are some side effects associated with these medications and they need to be taken under medical monitoring with blood tests to detect side effects. It is my practice that DH should be treated with a gluten-free diet for life and use of drugs to get immediate relief in the short term. It is usually possible to get patient off the Dapsone after several months of a strict gluten-free diet.

The most common complication of DH is scarring which usually fades with time. Occasionally there can be secondary infection from scratching. There is probably a slightly increased chance of malignancy in those with DH who are not on a gluten-free diet. Several physical triggers are known to set off an attack of DH, especially exposure to iodides and bromides which are contained in household cleaners. A very good reference for DH is available from the GIG in Washington.

Iodine testing for DH: This is an old procedure used to create DH blisters. By applying a 30 percent solution of iodine as a patch, a DH outbreak can be created. This may be applicable in some patients when a biopsy is needed and no blisters are available.

Immunofluorescence: The indirect immunofluorescence test shows that the serum of a patient contains specific antibodies that bind to different areas of the epithelium. The direct immunofluorescence tests by a skin biopsy shows a specific diagnosis pattern of DH. Traditionally this biopsy is obtained from the buttocks. If no outbreaks are observed in this area, the biopsy is recommended for another area where the itching is observed. DH Drugs: The common drugs used to initially control the blisters are: Dapsone, Sulfoxone, and Sulfapyridine. Each one has different advantages/disadvantages or availability in the treatment of DH. Dapsone changes the life span of red blood cells from an average of 120 days to 30 days. Dapsone is known for possible hematologic changes as a common side effect.

The following are excerpts from a lecture given by Dr Lionel Fry at the 1984 AGM in London. Dr. Fry is a consultant dermatologist. The lecture is entitled: Recent Studies in Dermatitis Herpetiformis.

..we have looked at the records of 78 patients who have been attending our special DH clinic. The length of follow-up of these patients has ranged from 3 to 14 years (mean 7.4). All patients were offered a gluten-free diet as part of their treatment. However, only 42 patients have taken the diet......in only 23 patients was the diet absolutely strict, in another 17 there had been very occasional, but unintentional gluten intake, and in 2 there had been occasional but intentional intake. When these three groups of patients are compared it has been found that of the 23 patients taking a strict diet, 22 (96%) were able to stop drugs compared to 8 (47%) of 17 patients who had occasional but unintentional gluten (the 2 occasional but intentional gluten eaters could not stop drugs)........One of the most significant points to have emerged from our study is the time it takes with a gluten free diet before patients may reduce the dose of their drugs to control the rash, and eventually cease to need drugs. The mean time before there was a reduction in the dose of dapsone was 4-30 months (mean 8), and 6-108 months (mean 29 ) before the drugs were no longer required. These times were dependent on the strictness of the diet. ....In the past many doctors have been unaware that it has taken so long before the drugs could be reduced or stopped and this led to a situation where it was thought that the rash was not due to gluten.......Twelve of our patients agreed to take gluten again to see if their rash returned. These 12 patients had been on a gluten free diet for periods ranging from 3-12 years (mean 7.5). In 11 of the 12 patients the rash recurred in times ranging from 2-36 weeks (mean 12). It could be argued that in the patient whose rash did not recur had undergone spontaneous remission........ (sections of the text of a talk by Dr. Lionel Fry, Consultant Dermatologist, St Marys Hospital, London W2).

Dermatitis Herpetiformis Summary

A dermatologist who is experienced at recognizing dermatitis herpetiformis should do the biopsy. The biopsy is taken of one of the blisters or the skin at the edge of the lesion. The biopsy should not be taken from the lesion, but from the edge or just near the lesion - it can be misdiagnosed as herpes if taken from the lesion. An iodine patch can be used to bring about a blister. If one has dermatitis herpetiformis, a blister will form; if not, one does not have dermatitis herpetiformis. A positive dermatitis herpetiformis biopsy will show IgA antibodies. The lab should be looking for IgA deposits in a granular line at a specific location in the skin. Some dermatologists use an immunofluorescence method of examination. dermatitis herpetiformis usually appears where pressure is applied to the body, but can appear anywhere. If the biopsy is not taken correctly you can get an incorrect negative. This is a positive method of diagnoses.

• Iodine can trigger eruptions in some people (with dermatitis herpetiformis). However, iodine is a essential nutrient and should not be removed from the diet without a physicians supervision.
• Iodine does not contain gluten. Iodine can worsen the symptoms of skin lesions in patients with dermatitis herpetiformis.
• When the deposits of IgA have been cleared from the skin over time by following a gluten free diet, iodine should no longer present any problem for dermatitis herpetiformis patients.

As background, for those who are not familiar with Dermatitis Herpetiformis, the following description comes from a resource guide of the Gluten Intolerance Group of North America:

• Dermatitis herpetiformis (dermatitis herpetiformis) is a chronic disease of the skin marked by groups of watery, itch blisters. The ingestion of gluten (the proteins gliadin and prolamines contained in wheat, rye, oats, and barley) triggers an immune system response that deposits a substance, IgA (immonuglobin A), under the top layer of skin. IgA is present in affected as well as unaffected skin. dermatitis herpetiformis is a hereditary autoimmune disease linked with celiac disease. If you have dermatitis herpetiformis, you always have celiac disease. With dermatitis herpetiformis the primary lesion is on the skin rather than the small intestine. The degree of damage to the small intestine is often less severe or more patchy then those with only celiac disease. Both diseases are permanent and symptoms/ damage will occur after comsuming gluten.

When my husband was diagnosed with dermatitis herpetiformis last November, he went to visit a expert in dermatitis herpetiformis, Dr. John J. Zone, at the University of Utah (USA). The written instructions Dr. Zone gave him included the following statement:

• The mineral iodine is known to make the disease (dermatitis herpetiformis) worse. For this reason, foods and supplements high in iodine should be avoided. Table salt which is not iodized should be used. This can be found in most grocery stores with the other salts. Avoid kelp and other seaweed products, and do not use sea salt. If you take any nutritional supplements, examine them carefully to avoid any iodine containing ingredients.

It is not necessary for dermatitis herpetiformis patients to eliminate iodine completely from their diet, merely to avoid foods high in iodine as described above. Dr. Zone also explained that dermatitis herpetiformis patients need not avoid iodine indefinitely. Iodine is an important mineral for our bodies. dermatitis herpetiformis patients can stop avoiding iodine when their rash symptoms clear up which can take anywhere from a few months to a couple of years on a gluten-free diet.

More about iodine:

• Intake of large amounts of inorgana iodide is known to exacerbate symptoms and a few patients have been reported to improve on low iodide diets. However, this is not a mainstay of treatment and need only be considered if patients are consuming excessive iodide in the form of vitamin pills, kelp, or seafood. Likewise, some patients have reported exacerbation with thyroid hormone replacement therapy and thyrotoxicosis. In such cases, excessive thyroid replacement should be avoided and thyrotoxicosis treated appropriately.

• Dermatitis Herpetiformis, John J. Zone MD, Curr Probl Dermatol, Jan/Feb 1991, p36
• Dermatitis Herpetiformis is considered a rare skin disease.
• The true incidence and prevalence of dermatitis herpetiformis appears to vary in different areas of the world and may vary within the same country. During 1987, 158 cases of documented dermatitis herpetiformis were identified in the state of Utah out of a population of 1.6 million, a prevalence of 9.8 per 100,000.
• Dermatitis Herpetiformis, John J. Zone MD, Curr Probl Dermatol, Jan/Feb 1991, p15