Diagnosed with gluten intolerance; is endoscopy needed with twice confirmed biopsy for DH and positive blood panel

[cstutz]

I am fairly new to all of this, and I apologize if my questions are redundant. I think I have had gluten-related symptoms, such as: abdominal pain, bloating, diarrhea in the early years followed by extreme constipation for last 5-10yrs, anemia, numerous vitamin deficiencies, migraines, joint pain, etc, since at least my early teens (now 39). For as long as I can remember I have had “eczema” and been lactose intolerant. About nine months ago, things went a little crazy after I developed a rash on my one breast. Mammogram, breast ultrasound, numerous rounds of antibiotics later and I was finally referred to a dermatologist.
As I said, I have had “eczema” for most of my life. I had rashy areas on my knees, elbows, scalp, abdomen, buttocks, fingers; and, of course, on my breast; however, I did not connect the rash on my breast to the other areas, as it seemed different and had come about years after the other rashy areas. From there, I had biopsies from multiple areas of my skin to check for allergic reaction vs psoriasis vs dermatitis herpetiformis vs lupus. I was prescribed a topical ointment to apply to the rash while awaiting the biopsy results. A few weeks later, I had more biopsies taken for direct immunofluorescence, which were consistent with dermatitis herpetiformis.
This is where things became a little murky for me. My doctor recommended that I have bloodwork drawn for a celiac panel of testing; even though, I was, as she put it, “asymptomatic.” The results of that testing: Deamidated Gliadin Abs, IgA units: 76 (negative: 0-19), (weak positive: 20-30), (moderate to strong positive: >30). t-Transglutaminase |gG U/mL: 2 (negative: 0-5), (weak positive: 6-9), (positive: >9). IgA, Qnt, serum mg/dL: 59 (normal: 87-352). Deamidated Gliadin Abs, IgG units: 92 (negative 0-19), (weak positive: 20-30), (moderate to strong positive: >30). t-Transglutaminase |gA U/mL: 15 (negative: 0-3), (weak positive 4-10), (positive: >10). Tissue Transglutaminase (tTG) has been identified as the endomysial antigen. Studies have demonstrated that endomysial IgA antibodies have over 99% specificity for gluten sensitive enteropathy. Endomysial IgA: Positive (Standard Range Negative). Given the lab results and the skin biopsy results both being indicative of celiac disease, is an endoscopy needed for further confirmation? Also, can constipation be a symptom, or only diarrhea? I think my doctor labeled my as “asymptomatic” because I had constipation. Interestingly enough, after having been gluten free for a few months, I’m not nearly as constipated and my DH rash has improved somewhat. 
Thank you in advance! 

 

[trents]

Welcome to the forum, cstutz!

First, DH is definitive for celiac disease. There is no other known cause of DH.

Second, most all of your antibody tests were strongly positive, despite your total IGA being low. Low total IGA will lower the numbers of individual IGA antibody tests.

You have many classic symptoms of celiac disease, including constipation.

Your symptoms have improved as you have removed gluten from your diet.

I am not sure what it is that is "murky" about all this.

[cstutz]

  On 10/26/2022 at 6:40 PM, trents said:

Welcome to the forum, cstutz!

First, DH is definitive for celiac disease. There is no other known cause of DH.

Second, most all of your antibody tests were strongly positive, despite your total IGA being low. Low total IGA will lower the numbers of individual IGA antibody tests.

You have many classic symptoms of celiac disease, including constipation.

Your symptoms have improved as you have removed gluten from your diet.

I am not sure what it is that is "murky" about all this.

Expand Quote  

Sorry, if I wasn’t super clear. I will try to explain my confusion better because, from my perspective, it seems like a no-brainer that my diagnosis should be celiac disease, not gluten intolerance.

My doctor said I could have DH without having celiac disease, so, as she explained to me, an intolerance or sensitivity to gluten, but not celiac disease. This is why I was wondering if the endoscopy would still be needed to confirm a diagnosis of celiac disease with both the DH confirmed by biopsy and the positive blood panel. In the research I’ve done, I’ve found conflicting information about whether it was possible to have DH without also having celiac disease. However, I thought my bloodwork was strongly indicative of celiac with GI involvement, which would make the DH alone vs celiac disease moot. I also was confused by my doctor labeling me as asymptomatic because of the constipation, since, as she explained, celiac presents most commonly with diarrhea; however, I thought constipation could also be a symptom. She also said if I found gluten avoidance difficult or impossible, she could prescribe dapsone for the DH; however, I thought gluten ingestion was a definite no-go for the rest of one’s life with celiac disease. I guess what I find murky is being labeled as asymptomatic and diagnosed with gluten intolerance/sensitivity vs celiac disease when my bloodwork is strongly indicative of celiac disease. Hopefully that makes sense.

On an aside, I think I read somewhere that it wasn’t all that uncommon to have a lower total IGA with celiac disease, but I can’t say for sure where I found that.

The following is what I received from my doctor: 
“DIF bx: The DIF biopsy was consistent with dermatitis herpetiformis, which is the rash that is associated with gluten sensitivity. We should do blood work for celiac disease even though you are asymptomatic. You can have one without the other, though. The treatment of choice is avoidance of all gluten.”
“Celiac panel: Even though you do not have Gl symptoms, all of your celiac tests were positive. The treatment of choice for this is also avoiding gluten. If you don't find that you can avoid gluten, there is a medication that we can prescribe called dapsone. It of course has side effects, so avoidance of gluten is the preferred treatment of choice.”

Can you have gluten sparingly, due to no damage to the small intestine, with an intolerance vs celiac disease? I’m just so confused by the diagnosis of gluten intolerance vs celiac disease after the testing (the official diagnosis is listed as gluten intolerance in my medical record, which may affect how my medical insurance processes and covers potential future testing and treatments) and being offered dapsone if I can’t avoid gluten when I understood ingesting any gluten with celiac disease to be incredibly dangerous and damaging. I’ve done so much research and finding conflicting information just adds to the confusion.

For those who have both celiac disease and DH, how long after being gluten-free did your skin symptoms resolve? My DH rash has only slightly improved over the last few months with being gluten-free (unfortunately, I think I’ve had some cross-contamination; though) and using a topical ointment. I know dapsone has some side effects, but the DH rash is especially awful.

So, from my perspective, it seems like a no-brainer that the diagnosis should be celiac disease and not gluten intolerance. I’m just not sure if the endoscopy to confirm is necessary or even the correct next step for diagnosis. Thank you! 

[trents]

First of all, let's discuss some terminology as I'm not sure you and your physician are using some terms the same way that I would and many others in the gluten disorders community.

On this forum, tend to use the term "gluten intolerant" as an umbrella term to refer to both celiac disease and NCGS (Non Celiac Gluten Sensitivity). NCGS is often referred to simply as "gluten sensitivity". With celiac disease, there is almost always damage done to the small bowel villi but this does not happen with gluten sensitivity. However, DH is not a manifestation of gluten sensitivity, it is a variant of celiac disease. Most people who have DH also have damage to the villi. A small percentage do not. 

NCGS is not to be taken lightly as even though it does not damage the small bowel villi it can damage other body systems. For instance, we get lots of reports on this forum from NCGS people who experience neurological damage from gluten. For example, "gluten ataxia". And some gluten disorder experts believe NCGS can be a precursor to celaic disease.

Realize also that small amounts of gluten may not produce discernible symptoms but still be causing low grade damage to villi.

Whether you have celiac disease or NCGS the antidote is the same, strict avoidance of gluten for life, or until some effective cure becomes available. There is a significant learning curve involved in learning to eat truly gluten free (as opposed to just lowering gluten consumption) but many people do it all the time. You first have to make up your mind that this is reality now and push into it.

[RMJ]

What type of doctor have you been seeing? Some don’t know a lot about celiac disease. You may want a new doctor.

I see a problem with what your doctor said: “dermatitis herpetiformis, which is the rash that is associated with gluten sensitivity.”  That phrase is missing a word.  DH is the rash associated with gluten sensitive enteropathy.  Gluten sensitivity is NOT the same thing as gluten sensitive enteropathy.  Gluten sensitive enteropathy IS celiac disease.  Enteropathy derives from the words for the gastrointestinal tract and pathology. People with gluten sensitivity do not have the damage (pathology) in their GI system.

Your doctor also said: “you can have one without the other.”  You can certainly have symptoms of one without the other, but the two types of symptoms (skin and GI) are basically two manifestations of the same autoimmune disease, which your antibody tests indicate you have.

Here is information on DH from the NIH (National Institutes of Health) which may answer your questions. I highlighted a few spots:

Dermatitis Herpetiformis

Dermatitis herpetiformis (DH) is a chronic, intensely itchy, blistering skin manifestation of gluten-sensitive enteropathy, commonly known as celiac disease. DH is a rash that affects about 10 percent of people with celiac disease. DH is found mainly in adults and is more common in men and people of northern European descent; DH is rarely found in African Americans and Asian Americans.

Symptoms

Dermatitis herpetiformis is characterized by small, clustered papules and vesicles that erupt symmetrically on the elbows, knees, buttocks, back, or scalp. The face and groin can also be involved. A burning sensation may precede lesion formation. Lesions are usually scratched off by the time a patient comes in for a physical exam, and the rash may appear as erosions and excoriations.

Patients with DH may also experience dental enamel defects to permanent teeth, which is another manifestation of celiac disease. Less than 20 percent of people with DH have symptoms of celiac disease.

Causes

Dermatitis herpetiformis is caused by the deposit of immunoglobulin A (IgA) in the skin, which triggers further immunologic reactions resulting in lesion formation. DH is an external manifestation of an abnormal immune response to gluten, in which IgA antibodies form against the skin antigen epidermal transglutaminase.

Family studies show that 5 percent of first-degree relatives of a person with DH will also have DH. An additional 5 percent of first-degree relatives of a person with DH will have celiac disease. Various other autoimmune diseases are associated with DH, the most common being hypothyroidism.

Diagnosis

A skin biopsy is the first step in diagnosing DH. Direct immunofluorescence of clinically normal skin adjacent to a lesion shows granular IgA deposits in the upper dermis. Histology of lesional skin may show microabscesses containing neutrophils and eosinophils. However, histology may reveal only excoriation due to the intense itching that patients experience.

Blood tests for antiendomysial or anti-tissue transglutaminase antibodies may also suggest celiac disease. Blood tests for epidermal transglutaminase antibodies are positive in more than 90 percent of cases. All of these tests will become negative with prolonged adherence to a gluten-free diet.

A positive biopsy and serology confirm DH and should be taken as indirect evidence of small bowel damage. A biopsy of the small bowel is usually not needed for DH diagnosis. However, if clinical signs of gastrointestinal disease are evident on examination, further workup may be required. Whether or not intestinal damage is evident, a gluten-free diet should be implemented because the rash of DH is gluten sensitive.

Treatment

The sulfone dapsone can provide immediate relief of symptoms. For patients who cannot tolerate dapsone, sulfapyridine or sulfamethoxypyridazine may be used, although these medications are less effective than dapsone. A strict gluten-free diet is the only treatment for the underlying disease. Even with a gluten-free diet, medication therapy may need to be continued from a few months to 2 years.

[cstutz]

  On 10/27/2022 at 10:26 PM, trents said:

First of all, let's discuss some terminology as I'm not sure you and your physician are using some terms the same way that I would and many others in the gluten disorders community.

On this forum, tend to use the term "gluten intolerant" as an umbrella term to refer to both celiac disease and NCGS (Non Celiac Gluten Sensitivity). NCGS is often referred to simply as "gluten sensitivity". With celiac disease, there is almost always damage done to the small bowel villi but this does not happen with gluten sensitivity. However, DH is not a manifestation of gluten sensitivity, it is a variant of celiac disease. Most people who have DH also have damage to the villi. A small percentage do not. 

NCGS is not to be taken lightly as even though it does not damage the small bowel villi it can damage other body systems. For instance, we get lots of reports on this forum from NCGS people who experience neurological damage from gluten. For example, "gluten ataxia". And some gluten disorder experts believe NCGS can be a precursor to celaic disease.

Realize also that small amounts of gluten may not produce discernible symptoms but still be causing low grade damage to villi.

Whether you have celiac disease or NCGS the antidote is the same, strict avoidance of gluten for life, or until some effective cure becomes available. There is a significant learning curve involved in learning to eat truly gluten free (as opposed to just lowering gluten consumption) but many people do it all the time. You first have to make up your mind that this is reality now and push into it.

Expand Quote  

Thank you so much for the replies and clarification! I do find the terminology confusing, so thank you for explaining the different terms. You said, “However, DH is not a manifestation of gluten sensitivity, it is a variant of celiac disease.” From the way my doctor explained things, I was under the impression that DH was something different than celiac disease and that it, along with gluten intolerance/sensitivity, were less severe. That mistaken impression together with her statements about being gluten-free as the treatment of choice with dapsone as an option if I couldn’t “avoid gluten,” really made it seem like a strict gluten-free diet was somewhat optional. 

This may be a silly question, but can DH be due to topical gluten exposure or only from ingesting gluten? Reading product ingredient labels has been quite the eye-opening experience. Wow, I had no idea how many products contain gluten! 

[cstutz]

  On 10/28/2022 at 1:41 AM, RMJ said:

What type of doctor have you been seeing? Some don’t know a lot about celiac disease. You may want a new doctor.

I see a problem with what your doctor said: “dermatitis herpetiformis, which is the rash that is associated with gluten sensitivity.”  That phrase is missing a word.  DH is the rash associated with gluten sensitive enteropathy.  Gluten sensitivity is NOT the same thing as gluten sensitive enteropathy.  Gluten sensitive enteropathy IS celiac disease.  Enteropathy derives from the words for the gastrointestinal tract and pathology. People with gluten sensitivity do not have the damage (pathology) in their GI system.

Your doctor also said: “you can have one without the other.”  You can certainly have symptoms of one without the other, but the two types of symptoms (skin and GI) are basically two manifestations of the same autoimmune disease, which your antibody tests indicate you have.

Here is information on DH from the NIH (National Institutes of Health) which may answer your questions. I highlighted a few spots:

Dermatitis Herpetiformis

Dermatitis herpetiformis (DH) is a chronic, intensely itchy, blistering skin manifestation of gluten-sensitive enteropathy, commonly known as celiac disease. DH is a rash that affects about 10 percent of people with celiac disease. DH is found mainly in adults and is more common in men and people of northern European descent; DH is rarely found in African Americans and Asian Americans.

Symptoms

Dermatitis herpetiformis is characterized by small, clustered papules and vesicles that erupt symmetrically on the elbows, knees, buttocks, back, or scalp. The face and groin can also be involved. A burning sensation may precede lesion formation. Lesions are usually scratched off by the time a patient comes in for a physical exam, and the rash may appear as erosions and excoriations.

Patients with DH may also experience dental enamel defects to permanent teeth, which is another manifestation of celiac disease. Less than 20 percent of people with DH have symptoms of celiac disease.

Causes

Dermatitis herpetiformis is caused by the deposit of immunoglobulin A (IgA) in the skin, which triggers further immunologic reactions resulting in lesion formation. DH is an external manifestation of an abnormal immune response to gluten, in which IgA antibodies form against the skin antigen epidermal transglutaminase.

Family studies show that 5 percent of first-degree relatives of a person with DH will also have DH. An additional 5 percent of first-degree relatives of a person with DH will have celiac disease. Various other autoimmune diseases are associated with DH, the most common being hypothyroidism.

Diagnosis

A skin biopsy is the first step in diagnosing DH. Direct immunofluorescence of clinically normal skin adjacent to a lesion shows granular IgA deposits in the upper dermis. Histology of lesional skin may show microabscesses containing neutrophils and eosinophils. However, histology may reveal only excoriation due to the intense itching that patients experience.

Blood tests for antiendomysial or anti-tissue transglutaminase antibodies may also suggest celiac disease. Blood tests for epidermal transglutaminase antibodies are positive in more than 90 percent of cases. All of these tests will become negative with prolonged adherence to a gluten-free diet.

A positive biopsy and serology confirm DH and should be taken as indirect evidence of small bowel damage. A biopsy of the small bowel is usually not needed for DH diagnosis. However, if clinical signs of gastrointestinal disease are evident on examination, further workup may be required. Whether or not intestinal damage is evident, a gluten-free diet should be implemented because the rash of DH is gluten sensitive.

Treatment

The sulfone dapsone can provide immediate relief of symptoms. For patients who cannot tolerate dapsone, sulfapyridine or sulfamethoxypyridazine may be used, although these medications are less effective than dapsone. A strict gluten-free diet is the only treatment for the underlying disease. Even with a gluten-free diet, medication therapy may need to be continued from a few months to 2 years.

Expand Quote  

Thank you so much for the reply and your explanations! Your statement, “I see a problem with what your doctor said: “dermatitis herpetiformis, which is the rash that is associated with gluten sensitivity.”  That phrase is missing a word.  DH is the rash associated with gluten sensitive enteropathy.  Gluten sensitivity is NOT the same thing as gluten sensitive enteropathy.  Gluten sensitive enteropathy IS celiac disease,” was incredibly helpful and cleared up a few of my misconceptions. There is so much information out there, with plenty of it being in conflict, and it’s been difficult to wrap my brain around all of it! 
Thank you so much for your help as I begin to navigate all of this!

[trents]

DH and villi blunting of the small bowel lining are both manifestations of celiac disease. The vast majority of celiacs have villi blunting. A smaller percentage also have DH. Still a smaller percentage have only DH. We don't really know why there is this variation but suffice it to say we are all different and our immune systems will not all react in the same way.

Regardless of whether you have celiac disease or NCGS a strict gluten free diet should not be considered optional. Many with DH, despite their best efforts to eat gluten free, still have outbreaks occasionally but less often than before they started on the gluten-free diet. Some of those still do need to take Dapsone for consistent control of their DH. But taking Dapsone in lieu of a strict gluten-free diet should not be looked upon as an option in any case.

One thing thing we have come to realize in the celiac community is that there is an appalling knowledge vacuum in the medical community with regard to gluten disorders. Most physicians are operating on very outdated information and downright misinformation in some cases. We have learned from experience not to trust them in a cart blanche way in this area of medicine. We have learned that we have to go to appointments armed with knowledge and prepared to advocate for ourselves by being appropriately and respectfully assertive. That is often the only way we can get necessary testing ordered.

Concerning your question as to a topical gel containing gluten possibly eliciting a reaction like ingestion of gluten does, yes, there are some celiacs who experience reactions from topical exposure. Though I can't state with certainty the same is true for those with NCGS, it would not surprise me. We absorb many things through our skin to one degree or another and those in the most sensitive end of the spectrum of gluten disorders can react to transdermal exposure.

It's also helpful to know that some experts believe that NCGS can be a precursor to celiac disease. There is still a lot we don't know about the genetics of gluten disorders and the details of immune system pathways and interactions. It's not always as black and white as we would like. Some of is is still kind of murky yet.

[trents]

Technically speaking, RMJ is correct. Celiac disease is also known as "gluten sensitive enterophathy". But still, there are those with celiac disease who are the outliers and don't have enteric involvement but do have DH. I do not think it would be correct to isolate DH from celiac disease since the vast majority of suffers have both together. But that is my opinion.

Having said that, it is my observation is that in this forum community we typically use the term "gluten sensitivity" as equivalent to NCGS and Gluten intolerance to refer either celiac disease or NCGS. There is always confusion with terminology until all parties in a discussion are agreed on definitions. Just be aware that these terms are not used the same way by everyone and contain some elasticity. My suggestion to you, cstutz, is that you confine yourself to either using "celiac disease" or "NCGS"

[LCAnacortes]

I was getting lesions on my head - from a hair conditioner that contained wheat.  Be sure to check all of your personal care products for gluten and your pet food & care products too. Any exposure to gluten can cause a flare.  I haven't been diagnosed but have something that seems to be DH.  It went much better when I went gluten free back in May.  I have had cross contamination issues too.