Dermatitis herpetiformis, once considered celiac disease of the skin, is known to occur in a number of different autoimmune disorders.
Dermatitis herpetiformis is an autoimmune disorder characterized by chronic, intensely pruritic (itchy) symmetric groups of vesicles, papules, and wheals (hives), that may occur on the elbows, knees, arms, legs, shoulders, scalp, buttocks, neck, and face. Dermatitis herpetiformis, which is also known as Duhrings’s disease, Brocq-During disease, and dermatitis multiformis, usually occurs in people with celiac disease and well as milder forms of gluten sensitivity, and less often, in people with autoimmune thyroid disorders.
Dermatitis herpetiformis has a typical onset in the late teens and early twenties, or in the third or fourth decades of life, although it can affect people of all ages. Males are affected twice as often as females, and it occurs more often in whites than in people of Asian or African descent.
Symptoms
Dermatitis herpetiformis causes small blisters, discrete papules (bumps, pimples), itchy smooth lesions resembling hives, and waxy lesions. Blisters and papules frequently appear on the face and and may cause intense itching. Clinical signs are often highly variable ranging from groups of papulovesicles with excoriations or eczema-like lesions to minimal variants of discrete redness with small water blisters or areas of small purpura (purple bruise-like lesions).
Untreated, dermatitis herpetiformis tends to wax and wane although with a constant diet of gluten, symptoms persist. It can take a few weeks to several years for symptoms to clear with a gluten-free diet with longer periods required for patients who have had symptoms for a long time before restricting gluten.
Although lesions in the oral mucosa are rare, there are reports of oral lesions occurring early in the stages of dermatitis herpetiformis. These lesions may also be caused by the apthous ulcers (oral blisters) that frequently occur in people with celiac disease.
Environmental Triggers
Environmental triggers include gluten, which is found in wheat, rye, and barley, and other grains that are contaminated with wheat during harvest. Gluten is also found in hydrolyzed vegetable protein, artificial colorings, malts, malt ales, hydrolyzed plant protein, monosodium glutamate, preservatives, modified food starches, vegetable gum, beer, and vinegar. Iodide in iodized salt and foods high in iodine and halide are suspected of causing disease flares.
Diagnosis
Biopsy of the lesions in dermatitis herpetiformis shows dermal papillary microsabscesses of neutrophilic white blood cells with deposits of immunoglobulin A (IgA) and complement at the junction of the dermal and epidermal layers of the skin.
Treatment
Treatment for dermatitis herpetiformis includes: the antibiotics dapsone and sulfapyridine, and a gluten free diet. Dapsone effectively reduces the rash within several days. However, long-term use can result in anemia. A gluten-free diet generally limits the need for continued dapsone use. Often, dapsone is used initially to reduce inflammation and symptoms are controlled, often resolving after 18 months of a gluten-free diet. However, symptoms often return over time when gluten is resumed. In autoimmune thyroid disease, reduction of thyroid antibodies offers benefits. Recent studies show the effectiveness of selenium in reducing thyroid peroxidase (TPO) antibody titers.
Although dermatitis herpetiformis usually occurs for life once it appears, permanent remission is reported to occur in 10-20 percent of patients, usually after long-term adherence to a gluten-free diet.
Resources:
Dermatitis Herpetiformis, Dermatologic Disease Database, American Osteopathic College of Dermatology, accessed May 17, 2007.
Sharon Longshore and Kenneth Tomecki, Skin Signs of Systemic Disease, August 4, 2004, Medicine Index, The Cleveland Clinic.
Nino M, Ciacci C, and Delfino M, A long-term gluten-free diet as an alternative treatment in severe forms of dermatitis herpetiformis, J Dermatolog Treat, 2007; 18(1):10-12.
The copyright of the article Dermatitis Herpetiformis in Autoimmune Skin Disorders is owned by Elaine Moore. Permission to republish Dermatitis Herpetiformis in print or online must be granted by the author in writing.
At 4yrs. of age I was diagnosed with dermatitus herpetiformus, does this
mean I have celiac disease? nialarue@nc.rr.com Female age 53
Nov 18, 2008 2:07 PM
Elaine Moore :
Hi, Usually, dermatitis herpetiformis occurs in people with celiac
disease, but not always. It can occur in other autoimmune disorders such as
autoimmune thyroid disease and it can also occur on its own. Best, Elaine
Jan 6, 2009 4:59 AM
Guest :
Elaine, Have you incountered Dermatitus Herpetiformus in a long time
Alopecia Areata patient? I have Alopecia for 43 years and the last 20 it
has been Universalis (total scalp and body hair loss). Now I am dealing
with symptom that sound very much like Dermatitus Herpetiformus.
Jan 6, 2009 9:22 AM
Elaine Moore :
Hi, Dermatitis herpetiformis can occur in a number of autoimmune
conditions besides celiac disease. See this link
http://www.ncbi.nlm.nih.gov/pubmed/9115907?ordinalpos=10&itool=EntrezSy
stem2. PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDo
cSum
I found this doing a search on alopecia and DH on Pub
Med.
This article describes the alopecia occurring in 1.6% of
cases of DH. Another abstract from an Indian journal describes 2
sisters having both alopecia and DH. Often, many years go by before a
second or third autoimmune disorder develops. You might want to search
some more on pub med for skin conditions, but your intuition is probably
right. Best, Elaine
Mar 4, 2009 5:53 PM
Guest :
Hi I had a rash a few years ago that appeared and my doctor put me on
prenidsone it cleared up, but came back in a few weeks. Then another doctor
put me on amoxcillin, for a sinus infection, which actually turned out to
be a abscess tooth, the amoicellin cleared up the rash. Now a few years
later the rash has reappeared, and now I know I have hypothyroidism could
this rash be connected ? I went to family doctor 2 weeks ago he put me on
Triamcinolone Acetonide which had no effect on it. The rash has gotten
worse and is on my arms, and shins, only. The rash looks similiar to
shingles but doesnt itch or anything just looks bad. Today I went back to
doctor and he now says I have autoimmune dermatitis which is what lead me
to your site. Is there a chance this is what I have since I have a Thyroid
condition ? Thanks Mike
Mar 5, 2009 7:41 AM
Elaine Moore :
Hi Mike, It's possible to have dermatitis herpetiformis when you have
a thyroid condition. You might want to try eliminating wheat, rye, and
barley to see if you rash goes away. It can take several weeks. If you do
have dermatitis herpetiformis, you'd want to look into following a
gluten-free diet. It's not as hard as it first seems. Here's a link that
will take you to several other articles I've written on autoimmune skin
disorders on this website, autoimmune-skin-disorders.suite101.com/ Best, Elaine
Apr 30, 2009 12:41 PM
Elaine Moore :
Hi, People with vitiligo are likely to have another autoimmune
disorder, and celiac disease is a good possibility. Considering the rash
you have, dermatitis herpetiformis is even more likely. Blood tests for
certain antibodies (gliadin, endomysial, tissue transglutaminase) can be
used to diagnose celiac disease and dermatitis herpetiformis. Best,
Elaine
May 5, 2009 7:06 AM
Elaine Moore :
It's possible but since your rash seems to be related to heat and sun it's
probably not the first thing that comes to mind. It would be good to see a
dermatologist or an internist. It would be easy enough to run the blood
tests that are used to diagnose dermatitis herpetiformis. best to you,
elaine
Sep 1, 2009 12:14 PM
Elaine Moore :
Hi, Since you were already diagnosed with celiac disease, it's very
likely that your rash is caused by dermatitis herpetiformis. This rash
usually accompanies celiac disease although it can also occur in autoimmune
thyroid and other disorders. Avoiding wheat, rye, and barley should help
with the rash. Best, Elaine
Sep 14, 2009 10:08 AM
Elaine Moore :
Hi, It could be that the rash on your face and scalp isn't from DH but
has another cause. It's also possible that you have DH that is related to a
different autoimmune condition besides celiac disease. For instance,
autoimmune thyroid disorders can be accompanied by DH. Here, you would want
to lower thyroid antibody levels. Have you been tested for thyroid
antibodies? It usually takes at least 3 months to clear out all
gluten from your system. You might also try being more diligent about
avoiding it and try it for a longer period. Best, Elaine
Sep 15, 2009 11:46 AM
Guest :
Hello, I'm the person re: the facial/face rash... thank you very much for
your reply... I haven't been tested for thyroid antibodies, or anything
else; this rash began about 2 yrs ago and has gotten more persistant
lately, last time I went w/ my dr. (about 10 mos.) they just did general
lab work and every thing came back fine. I am about to go to my dr. again
and I will mention all of my symptons. I did some web research on auto
immune thyroid disorders, and I do have some other symptons as well, as I
told you before I am 37, and I also experience a warm overall sensation
when the temperature is fine (I'm always hot), my eyes seem to be red (they
are not clear), I feel a bit of arthritis in my hands, and I have started
getting alot of white hair (both my parents had almost balck hair into
their early fifties). I plan to start a gluten-free diet again after I
check in w/ my dr., and this time I plan to prepare myself better for a
longer period (2-3 mos) to see if I notice a difference. I know that every
body is different, but I have read cases that in some people the response
is faster. Thank you again for your feedback, it really helps!