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Celiac Disease and Skin

 

Celiac disease, both diagnosed and undiagnosed, can affect your skin. To date, a multitude of skin disorders have been associated with celiac disease, including:

  • Alopecia areata (hair loss)
  • Aphthous stomatitis (canker sores)
  • Atopic dermatitis (Eczema)
  • Chronic ulcerative dermatitis (associated with bacterial growth)
  • Cutaneous vasculitis (associated with inflammation)
  • Dermatomyositis (a type of inflammatory myopathy)
  • Dermatitis herpetiformis (DH)
  • Oral lichen planus (chronic mouth sores)
  • Prurigo nodularis (associated with autoimmunity)
  • Psoriasis
  • Urticaria (hives)
  • Vitiligo (pale patches of skin)

NFCA wants to help you keep your skin looking healthy and beautiful. So, we bring you this two-part series: Celiac and Skin. First, learn about the classic celiac skin issue, dermatitis herpetiformis. In August, return for Part 2, when we explore the relationship between celiac, psoriasis and eczema.

[Note: Click on the photos to see a larger version. Click on the document icon for a printable handout on the topic.]

Celiac & Skin: Part 1

Dermatitis Herpetiformis

What is DH? Who gets it?Dermatitis Herpetiformis handout

Dermatitis Herpetiformis, DH, is a painful skin rash that manifests as a direct result of untreated or undiagnosed celiac disease. It is commonly known as “celiac disease of the skin.”

About 15-25% of individuals with celiac disease experience DH, and men are affected more than women by a ratio of 2:1.

While DH can appear at anytime, the average age of onset is between 15-40 years of age.  

What does it look like?

o  Symptoms

o  Related Conditions

The hallmark sign of DH is an intensely itchy, blistering skin rash.Dermatitis Herpetiformis close up

Reddened skin, small circular bumps, and blisters filled with clear, neutrophil (white blood cell) containing liquid are very common.  Skin lesions and scarring can also appear, particularly in patients that scratch and irritate the skin during outbreaks. 

DH can appear anywhere on the body; however, it most frequently presents in the following areas:

  • Buttocks
  • Elbows
  • Knees
  • Lower back
  • Scalp

back rash due to celiacThe onset of DH may be acute or gradual, appearing in the same location every time. DH outbreaks are very often mirrored, meaning that the rash will occur on both sides of the body in the exact same place.

While DH is a known symptom of celiac, many patients with DH will not develop any classic digestive symptoms. This particular skin manifestation often does not correlate with a positive celiac diagnosis via biopsy. In fact, up to 20% of patients actually have normal small intestines when examined.

Some patients may exhibit signs of celiac, such as anemia or osteoporosis, at the time of their diagnosis.

Regardless of the presentation or symptoms, a positive diagnosis of DH always indicates celiac disease!

Because DH is an autoimmune condition, patients with the skin disorder can develop similar, more serious conditions like cancer if left untreated. Autoimmune thyroid diseases occur in nearly 20-30% of DH cases.

What causes DH?

Not much is known about what causes DH, but doctors believe genetic predisposition, heightened immunological responsiveness, and environment (i.e. gluten exposure), often in combination, play a role.

How is it diagnosed?

o  Skin Biopsy

o  Misdiagnoses

Dermatitis Herpetiformis on elbowsWhile 70-80% of DH patients have higher than normal blood IgA- tTG antibody levels, a typical celiac panel (blood test) is not considered sufficient or reliable enough to properly diagnose patients.

Instead, doctors diagnose DH by examining the dermal papillae (cells under the top layer of skin), and use a process called direct immunofluorescence to detect for neutrophils and granular IgA deposits in the skin.

Granular IgA deposits (which indicate DH) appear in a very distinct pattern when inspected via immunofluorescence and appear in 98% of patients with DH - making it the gold standard in gaining a diagnosis.

These types of skin samples are collected by performing a biopsy, which involves incising tiny portions of unaffected skin positioned immediately next to reddened or blistered areas.

Inflammation and blistering of skin (likely caused by itching or scratching) can alter the look and concentration of the IgA deposits present in patients with DH, affecting the patient’s ability to receive a proper diagnosis. Because of this, it’s very important that unaffected skin be collected during a skin biopsy.

DH can often be misdiagnosed and frequently confused with skin conditions such as: allergies, bug or mosquito bites, contact dermatitis, diabetic pruritus, eczema, herpes, hives and psoriasis.

The lesioning of individual blisters can often lead to this misdiagnosis, as scarring can cause a change in presentation, making it difficult for doctors to differentiate DH from other skin conditions.

Treatment

o  Gluten-Free Diet

o  Dapsone

o  Alternative Temporary Therapies

Like celiac disease, DH is a chronic autoimmune condition triggered by the consumption of gluten, and can only be treated through a lifelong gluten-free diet.

Skin contact with gluten containing foods and products has not been shown to cause DH outbreaks.

While a strict gluten-free diet will eventually eliminate both granular IgA deposits in the skin and the outward signs and symptoms of DH - it may take several weeks, even months, for skin to completely heal.

As a result, Dapsone oral medication is often prescribed to help eliminate the uncomfortable DH symptoms until the gluten-free diet takes full therapeutic effect.  Dapsone is used to suppress both symptoms and additional outbreaks, but cannot be used as an alternative treatment to the gluten-free diet. 

In general, itching and new lesions will begin to subside within 48-72 hours of starting Dapsone.

While well tolerated in 90% of patients, Dapsone does possess a variety of serious side effects. As a result, this therapy must be properly prescribed and vigilantly monitored by a doctor. 

Symptoms like nausea and upset stomach are often reported, so taking Dapsone with food is recommended for those who experience these problems.  Flu-like symptoms, fatigue and sensitivity to light are also common problems. More serious side effects include anemia, headaches, kidney damage, and peripheral neuropathy.

As a result, your doctor will most likely prescribe Dapsone in smaller doses when first starting out, and then increase the amount taken overtime.

What if I can’t tolerate Dapsone?

Topical treatments containing the steroid cortisone can also provide short-term relief from DH symptoms. Skin treatments that work to suppress the immune system are additional alternatives your doctor may recommend.

Like Dapsone, these topical remedies carry side effects and risks, and should only be prescribed as a secondary treatment for providing short-term relief from DH and taken under the supervision of a physician.

Remission

DH has been known, in some cases, to go into remission, whether or not patients are adhering to a gluten-free diet. Research indicates that DH remission is both spontaneous and only experienced in a very small percentage of patients (about 12%).

However, patients diagnosed with DH should NOT abandon a gluten-free diet at anytime, regardless of any apparent remission.

References:

  • Blumer, Ian, MD, Crowe, Shelia, MD. Celiac Disease for Dummies. Wiley & Sons, 2010.
  • Dennis, Melinda, MS, RD, LDN Leffler, Daniel, MD, MS. Real life With Celiac Disease. AGA Press, MD, 2010.
  • Green Peter H.R, Jones R. Celiac Disease: A Hidden Epidemic. HarperCollins, NY, 2010
  • NIH Consensus Development Conference on Celiac Disease, June 28th-30th, 2004.
  • Remission in Dermatitis Herpetiformis, Arch of Dermatol. 2011;147(3):301-305.

Related Content:

Part 2 »

 
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