Dermatitis herpetiformis usually presents with a pruritic skin rash. The lesions, vesicles and papules, will appear on the skin in groups and can be as big as one centimeter in diameter. These can occur anywhere on the skin, but the rash associated with the disorder usually effects the elbows, forearms, knees, buttocks, and scalp of the patient [7]. Other symptoms associated with the condition are burning, intense pruritus, and a stinging sensation which usually precede lesions.

The severity of the condition can vary rapidly since it is closely associated with a person’s diet. The majority of patients have an associated small bowel disease of varying degree related to gluten-sensitivity.

• Hodgkin Lymphoma

  The risk of non-Hodgkin's lymphoma is increased, but in patients with DH who adhere strictly to a gluten-free diet long-term prognosis is excellent. [ncbi.nlm.nih.gov]

  lymphoma (NHL)* affecting the intestines or any part of the body *The risk of NHL is increased among dermatitis herpetiformis patients. [dermnetnz.org]

  Malignancy Patients with dermatitis herpetiformis may be at higher risk of non-Hodgkin lymphoma (specifically enteropathy associated T cell lymphoma). G. Immunosuppression (HIV, chronic steroids, etc). No change in standard management. H. [clinicaladvisor.com]

• Weakness

  Dermatitis herpetiformis can be treated with antibiotics but there are side effects which can occur such as liver problems, light sensitivity, anemia, muscles weakness and peripheral neuropathy. [belmarrahealth.com]

  We performed another DIF, which was interpreted as DH by the same dermatolopathologist and tissue transglutaminase antibody testing was positive at 117 U (negative < 20 U, weak positive 20-30 U, strong positive > 30 U). [dermatology.cdlib.org]

  Most people see relief from taking dapsone, but side effects may include: liver problems sensitivity to sunlight anemia muscle weakness peripheral neuropathy Dapsone may also have negative interactions with other medications, such as aminobenzoate potassium [healthline.com]

• Fatigue

  We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: January 31, 2019 [patientslikeme.com]

  […] gluten (found in grains such as wheat, barley and rye) in their diet GI symptoms Although 90% of patients will have associated coeliac disease only a few are symptomatic, conversely 15-25% of patients with coeliac disease develop DH Symptoms include fatigue [pcds.org.uk]

  Sometimes, these symptoms may be accompanied by symptoms of coeliac disease, commonly including abdominal pain bloating or loose stool and fatigue. The rash caused by dermatitis herpetiformis forms and disappears in three stages. [webdicine.com]

  Sometimes, these symptoms may be accompanied by symptoms of coeliac disease, which typically include abdominal pain, bloating or loose stool, weight loss, and fatigue. [en.wikipedia.org]

• Short Stature

  Childhood Children with celiac disease may present with short stature, anemia, hepatitis, epilepsy and other extragastrointestinal conditions. With age, these presentations become more subtle. [aafp.org]

• Diarrhea

  Patients with GSE may present with hypokalemia in association with diarrhea and emesis, and if potassium loss is rapid, rhabdomyolysis may occur. [ncbi.nlm.nih.gov]

  Gastrointestinal system Diarrhea in 60 percent of patients (small intestinal type), early diarrhea occurs with infrequent, large, watery, foul stools; later diarrhea occurs more frequently Constipation in 20 percent of patients, with occasional obstipation [aafp.org]

  The symptoms of childhood CD include persistent diarrhea, failure to thrive, abdominal pain, and vomiting. Iron deficiency anemia also may be present as well as other sequelae of malabsorption. [mdedge.com]

  The common symptoms are foul-smelling diarrhea, bloated abdomen, and anemia. Causes Dermatitis herpetiformis is idiopathic (without known cause), but several immunologic abnormalities have been detected. [healthcentral.com]

• Wheat Intolerance

  Medical research suggests that in Dermatitis Herpetiformis wheat intolerance is the main cause. This disease is caused when gluten in the intestinal tract sticks to some antibodies and starts spreading in the bloodstream. [primehealthchannel.com]

• Blister

  On some occasions, the itchy sensation appears well before the blisters begin to the form. The blisters are full of a watery substance, and the blisters may weep in more severe outbreaks. [gluten-intolerance-symptoms.com]

  The blisters vary in size from very small up to 1 cm across. Treatment Dapsone is the drug of choice. [doctoralerts.com]

  Definition / general Uncommon autoimmune blistering disorder associated with gluten-sensitive enteropathy; very pruritic ( eMedicine, J Am Acad Dermatol 2011;64:1017 ) Involves extensor surfaces of elbows, knees, buttocks and back; also chest, axillae [pathologyoutlines.com]

  Dear Editor, Dermatitis herpetiformis (DH) is a chronic, polymorphic, pruritic autoimmune blistering skin disease characterized by subepidermal blisters, neutrophilic microabscesses, and granular IgA deposition within the dermal papillae. [ncbi.nlm.nih.gov]

• Eruptions

  The itching and burning of the eruptions are severe and the urge to scratch them is intense. Scratching will further irritate the eruptions. [gluten.org]

  This condition is sometimes misdiagnosed clinically as dyshidrotic eczema, contact dermatitis, scabies, and drug eruptions. [symptoma.com]

  We describe a 46-year-old man with DH who presented with the classic pruritic papulovesicular eruption and associated volar finger and palmar petechiae. We discuss recent advances in the pathogenesis and treatment of DH. [ncbi.nlm.nih.gov]

  The appearance of vesicobullous eruptions and excoriated prurigo–like eruptions can delay the diagnosis of DH for several months or years. [cfp.ca]

• Skin Disease

  Discussion Association of DH with various other autoimmune diseases and immune system mediated skin diseases have been described in various reports. [ijdvl.com]

• Papule

  D) Erythematous papules and urticarial lesions on the elbows. E) Excoriated papules and plaques on the submandibular area. [cfp.ca]

  On physical examination, the skin lesions manifested as erythematous papules, vesicles, and scabs on the limbs (Figure 2. a-c). She felt apparently pruritic. [ncbi.nlm.nih.gov]

  Dermatitis herpetiformis is a skin condition characterized by extremely itchy papules and vesicles usually on the extremities and trunk. It is often associated with a gluten-sensitive enteropathy (celiac disease). [clinicaladvisor.com]

• Eczema

  Sometimes a particularly stubborn case of DH may indicate the additional presence of atopic eczema. [gluten-intolerance-symptoms.com]

  What is eczema. Eczema Canada website. http://www.eczemacanada.ca/en/What-Is-Eczema. Accessed April 20, 2018. McKusick VA, ed. Online Mendelian Inheritance In Man (OMIM). The Johns Hopkins University. Dermatitis Herpetiformis, Familial. [rarediseases.org]

  The rash can look like eczema. Scratch marks and skin erosions instead of blisters in some people. Most people with DH have damage to their intestines from eating gluten. But only some have intestinal symptoms. [nlm.nih.gov]

  Still, DH is often confused with eczema, a common inflammatory skin disorder that, like DH, results in an itchy rash that is often scratched raw. [niddk.nih.gov]

• Genital Lesions

  And men are more likely to have atypical oral or genital lesions. The unusual outbreaks of DH on Rory Jones’ forehead and eyelids may have led her dermatologists down the wrong path. [niddk.nih.gov]

A proper diagnosis of dermatitis herpetiformis can only be made after laboratory tests, including tissue pathology, direct immunofluorescence microscopy, and serology. Using direct immunofluorescence, granular deposits of IgA within the dermal papillae will be revealed. This will exclude differential diagnoses such as bullous pemphigoid, herpes gestationis, linear IgA bullous dermatosis, and bullous systemic lupus erythematosus. This condition is sometimes misdiagnosed clinically as dyshidrotic eczema, contact dermatitis, scabies, and drug eruptions [8]. 

These tests are especially important after a clinically indistinguishable form of dermatitis known as gluten-sensitive dermatitis was documented in 2010. The one difference between the two conditions is the presence of IgA which is found in dermatitis herpetiformis but not gluten-sensitive dermatitis. 

While a gluten-free diet will improve the condition, the patient shouldn’t make any dietary changes before blood tests and skin biopsies are done. By changing a diet beforehand, any test results might show false negatives. If dietary changes were already made, it is recommended that a affected individuals reintroduce gluten into their diet for a few weeks before going for their tests. While doing this might make the patient unwell and uncomfortable, this is the only way to ensure that the tests will have reliable results.

The best lifelong treatment for dermatitis herpetiformis is a strict gluten-free diet. This diet will [9]:

• Reduce the need for medication to control symptoms. The diet can possibly allow a person to stop Dapsone, a common medication used to treat this ailment.
• Reduce the enteropathy and improve nutrition and bone density.
• Reduce the risk of possibly developing additional autoimmune conditions and the occurrence of intestinal lymphoma.

In terms of drug treatment, Dapsone is usually the first choice. This medication can reduce the pruritus associated with lesions and rashes in one or two days. Precautions, including blood monitoring requirements are usually necessary while on Dapsone. Other possible medications that are used to treat dermatitis herpetiformis are:

• Ciclosporin
• Colchincines
• Heparin
• Nicotinamide
• Sulfapyridine
• Systemic steroids
• Tetracycline
• Ultra-potent topical steroids

Patients dealing with dermatitis herpetiformis should be aware that there are some medications that can exacerbate their condition such as non-steroidal anti-inflammatory drugs (NSAIDs) and iodides. The only safe NSAID, according to research, seems to be ibuprofen. 

While a patient can go into remission from dermatitis herpetiformis, usually due to dapsone and/or a gluten-free diet, the disorder is considered to be a lifelong condition [6]. When it is left untreated, dermatitis herpetiformis can lead to issues including anemia, vitamin deficiencies, and in some cases, malignancies.

The exact etiology of the disorder is unknown. The majority of cases is associated with a gluten-sensitive enteropathy (GSE). The condition seems to have a genetic predisposition with increased expression of the HLA-A1, HLA-B8, HLA-DR3, and HLA-DQ2 haplotypes and IgA deposits in the papillary dermis are present. 

Dermatitis herpetiformis is an uncommon disorder. Across the globe, the condition seems to affect about 10 people out of 100,000. The prevalence in the United States is reported to be 11.2 cases in 100,000 people [3]. Males are more frequently affected by the disorder. The age of onset is usually around the fourth or fifth decade, but it may also affect children and the elderly.

The pathogenesis of dermatitis herpetiformis is a complex interplay between genetics, autoimmune factors, and environment. It involves the creation of IgA antibodies to gluten-tissue transglutaminase (t-TG). This is probably caused by a gluten sensitivity combined with a high-gluten diet in genetically susceptible individuals [4]. These t-TG antibodies cross-react with epidermal transglutaminase (e-TG) [5]. Deposition of IgA and epidermal TG complexes in the papillary dermis cause the lesions of the condition.

According to studies, the condition is found predominantly in people with Northern European roots and is rare in people of Asian and African descendants, most likely due to the frequency of certain haplotypes.

This condition is usually due to a genetic predisposition and is considered to be unpreventable. The complications of the condition, however, can be prevented. By eliminating gluten from a person’s daily diet, flare ups of dermatitis herpetiformis can be prevented [10].

Originally recorded in 1884 by Dr. Louis Duhring of the University of Pennsylvania, dermatitis herpetiformis is an autoimmune disease that causes cutaneous eruptions in the affected person [1]. The disorder is linked to gluten-sensitive enteropathy (GSE) and is recognizable by inflammatory papules, vesicles and excoriations on the on the forearms, knees, scalp, or buttocks. 

Dermatitis herpetiformis, sometimes known as gluten rash or Duhring’s disease, is a chronic blistering skin disorder that presents itself as a skin rash coupled by itching, blisters, and a burning sensation. These rashes usually appear on the back, buttocks, elbows, knees, and the scalp. This is usually coupled with an underlying genetic predisposition like a gluten sensitivity.