Thursday, February 19, 2009

Dermatitis Herpetiformis - a.k.a. DH

The CSA defines Dermatitis Herpetiformis (DH) as an important associated disorder or complication of Celiac Disease which is manifested in the form of a skin rash. There is strong evidence that the changes in the intestinal mucosa and the immunologic findings in the majority of patients diagnosed with DH are identical with those found in Celiac Disease. Gluten has been found to have a close relationship with this skin rash. DH is often referred to as "Celiac Disease of the skin" while CD is referred to as "Celiac Disease of the gut." Click here for more information about the symptoms of DH (including pictures).

Typically, DH can be characterized as an intensely itchy skin eruption distinguished by the formation of small papules or vesicles. The patient may perceive having red bumps and blisters. The symptoms of intense burning, stinging and itching cannot be overemphasized. It is common for DH lesions to be symmetrically distributed on the extensor surfaces of the body: over the elbows, knees, buttocks, scalp, posterior, neck, sacral (lower end of the spinal column) and shoulder areas. The face and facial hairline are occasionally affected. And rarely, the lesions occur within the mouth. Although its severity may vary, it persists indefinitely and is a lifelong condition. There are only a few patients in whom the disease remits for long periods of time.

DH was formerly described in literature as Duhring's Disease. It has a typical onset in the teenage years or in the third or fourth decades of life. In the U.S., the presence of diagnosed cases is estimated to be about 1 in 10,000 with a male/female ratio of 2:1. It is more common in whites and rare in people of African or Asian descent.

I was already "lucky" enough to be 1 out of 133 to have Celiac Disease. Now I am even more "lucky" - 1 out of 10,000! From what I have read, it is pretty rare to have both CD and DH - somewhere around 20% of CD patients also have DH. I was officially diagnosed with DH on Wednesday by a Dermatologist although I had suspected my elbow/knee skin rash was DH for many months now. The included picture is what my elbows look like about a week after eating Gluten. Same thing on my knees. In another week, the small blisters (which are extremely itchy) will turn to scabs and then eventually fade away. Why can't my luck translate into winning the powerball lottery which has a 1 in about 200 million occurrence? Guess I am not that lucky.

Regardless, the treatment for Celiac and DH are the same - lifelong Gluten Free Diet - so really nothing new for me.

Some questions I asked my Dermatologist...

Why do I have the DH rash after being Gluten Free for 8 months? - The skin can take up to 2 years or more to recover from Gluten ingestion after going Gluten Free. The intestines recover much faster.

Are there any medications to assist with DH? - Dapsone can be taken for this disease but has serious side-effects and you must be closely monitored while taking it. Taking a super potent steroid ointment like Clobetasol for about 2 weeks seems a little safer and should help clear up the rash and prevent the itching and burning but following a GF diet is the recommended approach for treating DH.

Are there any related diseases to DH? - Thyroid disease is most commonly associated with DH. Other autoimmune disorders that people with CD are at greater risk to develop include Addison' s disease, autoimmune chronic active hepatitis, Alopecia Areata, Graves' disease, insulin-dependent diabetes mellitus (type 1), myasthenia gravis, scleroderma, Sjogren's syndrome, systemic lupus erythematosus, and thyroid disease. This is not a complete list. Thyroid diseases and diabetes are the two most commonly associated diseases. It is not uncommon to have other skin conditions as well.

Anybody out there have DH and want to share their experiences?

9 comments:

Fred said...

Zach,

Sorry to hear that you too have DH. I had onset of symptoms as a teenager and was properly diagnosed about 15 years later when my wife first heard of Celiac through a co-worker who had been diagnosed.

In 2006 I went to the global Celiac Symposium in NYC and spent some time with Dr. John Zone, one of the world experts on DH. He is based out of Salt Lake City at the University of Utah. He was one of the original physicians to advocate the Dapsone prescription (which I also follow). Fortunately, he does a lot of work with Dr. Russell Hall who runs the dermatology program at Duke med school and is a leading expert on dermal auto-immune disease. Dr. Hall's website is here; [http://www.dukehealth.org/physicians/AC8FCFB84CBDBBE885256DFD006A9314]

Like Celiac. DH is commonly misdiagnosed. As a teenager I received several courses of Accutane as they thought I had a severe acne. My issues presented on the scalp and arms and the lesions looked similar to Chicken Pox.

The Dapsone has been very helpful for me in managing the DH and Dr. Hall has several patients who follow that therapy. He also has several topical antibiotics that have been recommended for flare-ups.

One thing I did pick up at the Symposium and through continued discussions with Doctors familiar with Celiac is that the GI and dermal versions morph back and forth more than is recognized in the literature. I've found personally that I am more sensitive now on the GI side than I was in the past and have heard similar comments from others with DH. I've also heard of cases where patients with the GI expression have morphed over to the dermal expression.

The toughest thing about DH compared to classic GI Celiac is the lag in symptom expression. Because the reaction to gluten contaminated foods can be so long, it seems like we have to be even more focused on what we eat.

Regards,
Fred

The Gluten-free 'Dish' said...

This is a question for Fred or anyone else with DH: What is the lag time for your blisters?
Thanks,
Debbie

Fred said...

It can be as fast as 1-2 days to the initial outset. Once present they can persist for months without any treatment.

I've asked and looked for any studies that tries to measure the lag but haven't seen anything. If anyone knows of such a study please share.

Emily said...

Hi Zach,

This is my main reaction when I have gluten by accident. It sucks; I itch something awful for a good 24 hours, which makes it nearly impossible to sleep due to scratching, but after that, the itching seems to go away. Occasionally I will blister (which does take a little while to heal), but maybe that takes a larger quantity of gluten, so this is infrequent for me.

Also, I eat Trader Joe's GF pasta all the time, including at the meeting, and never have any reaction....just my 2 cents on that topic...

Take care, and thanks again for all you do!

KK said...

Hi Zach,
Wow, how excited am I to find your blog!! I've not officially been diagnosed with DH, but have been dealing with the exact same symptoms for the last 4 months. After multiple prescriptions for high dose antihistamines that don't work for the rash on my arms, lower legs, and the backs of my hands, I finally realized I may have a food allergy. Well, my good old Clinical Nutrition text (I'm a dietitian in training) led me to the suspicion that I had DH. A little more reading, 3 weeks on a gluten-free diet and a trip to the allergist have led me to believe this is indeed the case. Eating GF has made a big difference, with the exception of some true newbie mistakes.

So... now I'm on the path to getting diagnosed, but it's a tricky one. I've got a derm appt in JULY and have to go beg my way next week into getting a GI referral.

Your stats on 1/10 000 may explain why the doc's I've spoken to all looked very puzzled when they see me.

One of my friends has CD but her symptoms only last 24hrs TOPS. My itch comes on almost immediately and will last for 12-24 hrs and then persists. Once it stops itching, then it burns and I'm left with the spots.

Glad to know there's someone else out there.
To those on Dapsone, how have you found the side effects??

Zach said...

KK - check out the health network for a Dermatologist that is familiar with DH and can diagnose you.

http://glutenfreeraleigh.blogspot.com/search/label/Gluten%20Free%20Raleigh%20Blog%20Health%20Network

-Zach

KK said...

Hi Zach ,
Thanks for that link. I'm in Canada, so I'll have to poke around up here. But thanks, your site is FABULOUS!!

Club Veg said...

Hi all,
I have Celiac and have had some skin outbreaks for many years (about 14). Never diagnosed and now I'm wondering if DH. But they don't particularly itch, it's just that I have these outbreaks on my skin - could be back, abdomen, etc. They seem to come in clusters. Anyone else have this happen with no itching?
Thanks.

Rita Brhel said...

I haven't been formally dx, despite seeing several dermatologists and having CD of the gut and the still-controversial ataxia symptoms, but there is no doubt that this rash is triggered by gluten. For me, it comes on within a day of being glutened (not right away, at least 6 hours after the incident) but lasts for months. And during those months, it'll wax and wane in intensity. Eventually it goes away but leaves hyperpigmented spots. It started on my face, but after I went gluten-free, with each accidental gluten ingestion, the rash grew bigger until now it's on my face, scalp, back of my neck, arms, chest, and belly. I don't do Dapsone as I'm breastfeeding, and no longer respond to Clobetasol. If it gets really, really bad, I take Prednisone but this isn't something to take long-term if you can help it. It is maddening, tho.

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