Dermatitis Herpetiformis (Dr. Papp)
Ron Hoggan from CCA Calgary

Dr. Kim Papp, a dermatologist, spoke to us about dermatitis herpetiformis (DH).  Upon entering the room, he was faced with a "confounding variable".  Despite a prior request, there was no slide projector.  Apparently undaunted, he placed a chair on the table at the front of the room, where he placed his laptop computer, so most of us could see slides.  He then revealed that he was struggling with laryngitis.  Things were not shaping up well for him that morning.

Nonetheless, he made a good start by surveying the crowd to see who had what symptoms.  Then he talked about the history of DH, saying that as long as there have been people, there has been DH.  I wondered about that, since he later indicated that DH was a manifestation of gluten intolerance.

He said that typical lesions appear on elbows, knees, buttocks, scalp.  Oral lesions are rare.  He also said that DH eruptions, unless typical are likely to be missed.

Dhuring, in 1884 was the first to differentiate DH from similar diseases.  But it has only been since 1941 that DH was connected with wheat.

The development of immunoflourescencea, about 1967/68, allowed DH to be distinguished from linear IgA disease and has facilitated the accurate diagnosis of this condition since that time.

Then he talked about the need for physicians to suspect DH and take an appropriate biopsy, send the specimen to an appropriate lab, and request the correct test.  If all this is done, an accurate diagnosis is possible.  But he did express the notion that many physicians would not be likely to suspect it or be able to take an appropriate specimen.  Dermatologists are most qualified for performing and interpreting skin biopsies.

Dr. Papp indicated that DH is uncommon, but not rare and that men are more afflicted than women (the reason for a gender difference is unknown, perhaps hormonal); and that there is genetic linkage.  The genetically coded immune system markers, HLA-B8, HLA-DQw3, and HLA-DQw2 are frequently found in DH patients.

The initiating factors in flares, Dr. Papp indicated are: iodides, certain medications, and diet.

When asked about stress, he said that stress  makes many symptoms worse.  It is not clear whether the stress makes the actual symptoms worse, or stress worsens the patient's sense of symptoms.

He talked about anti-inflammatory drugs which  may cause a flare, but may be taken in reasonable quantities.  Iodized  salt is the most common source of iodine but is unlikely to produce a flare of DH unless significant quantities are consumed.

GLUTEN: Hw went on to tell us that everyone with DH is gluten intolerant, but not all those with gluten intolerance have DH.  He also said that if you look hard enough, you will find intestinal damage in all cases of DH.  He argued that eventually, if the patient is gluten-free, other triggers will be unable to cause a flare, because gluten underlies the DH condition.

The only test, he told us, that differentiates Dh for a linear IgA disease in immunoflourescence testing, yet they are very separate conditions with distinct causes.  He also said that there are other blistering diseases which may further confuse the issue.

The diagnosis is made on the basis of signs and symptoms, a skin biopsy and the microscopic examination of that biopsy with certain staining techniques.  Although DH is a form of gluten intolerance, he said that a small bowel biopsy is unnecessary for diagnosing it.  He showed slides of the granular IgA deposits in DH, and showed us how to differentiate the slides showing linear IgA from those of DH.

DH is apparently the result of antibodies to gliand, a protein in gluten.  People with sprue develop antibodies and deposit them under the skin.  It is a genetically predetermined condition, but, Dr. Papp speculated that not everyone with the predisposition will develop DH.  We don't know all the answers to that, but it occurs more frequently in families of DH patients.

Then he talked about the controversy with oats.  People with celiac disease who are challenged with gliands from wheat, rye and barley develop symptoms.  But the precise peptide sequences found in gliands from wheat, rye, and barley have not been isolated in oats.

His statement regarding the oat gliand was based upon a recent article published in the New England Journal of Medicine:  uncontaminated oats  are safe.

TREATMENT:  He first introduced medications which included: sulphones and sulphonamides.

Then he talked about diet.  He insisted that those who comply with the diet are the exception, not the rule.  He argued that it was much more difficult and at times unpalatable gluten-free diet.  He claims to have tried many gluten-free foods. and found them adequate but not as varied or as appealing as a regular diet.

He then spoke briefly about abnormalities in the blood, hemotytic anemia, gastrointestinal symptoms, neurlogical symptoms which may be complications of medications used to treat DH.

When he spoke about the complications of DH he mentioned skin problems, nutrient malabsorption associated with intestinal damage, adenocarcinoma and lymphoma.  He indicated that the latter was very rare.  When asked if he thought it useful to  screen first degree relatives for Celiac Disease, he suggested that there is no evidence that there would be any benefit in performing the tests.  Even if an affected individual were discovered, there is no clear supporting evidence that intervention is beneficial.  Thus screening the first degree relatives is not economical because even where there might be a reasonable diagnostic yield, few folks are likely to comply with the diet Additionally, the associated cancers are very rare, and there is no evidence that early intervention would be preventative