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Dermatitis Herpetiformis

Dermatitis herpetiformis (Duhring's disease) is a chronic idiopathic skin disorder characterized by groups of severely pruritic papulovesicular eruptions, often associated with gluten-sensitive enteropathy. Symptoms typically first appear in the early years of adulthood.


Presentation

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.

Shivering
  • The patient suffered fever, sweating, shivering, and multiple enlarged cervical lymph nodes. The diagnosis of anaplastic large cell lymphoma was confirmed by the histologic features of a biopsied cervical lymph node.[ncbi.nlm.nih.gov]
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]
Colic
  • […] study of 846 patients published in 2008, contradicted this relationship, such as found no increased risk of mortality in these patients. 30 Although 100% of patients with DH present sensitivity to gluten enteropathy, only a minority develop symptoms of colic[dx.doi.org]
Dyspepsia
  • We here examined the frequency and topography of chronic gastritis in 93 untreated DH subjects and in 186 controls with dyspepsia.[ncbi.nlm.nih.gov]
Hepatomegaly
  • However, 3 weeks after the start of DDS, he suffered from edematous eruption on the cheeks and neck, enlargement of the pharynx, systemic lymphoadenopathy and hepatomegaly.[ncbi.nlm.nih.gov]
  • Involvement of other organs varies and includes the liver (hepatomegaly, icterus, hepatitis and hepatic encephalopathy), lymphadenopathy, eosinophilia, and others. [ 46, 338, 339, 340 ] The course of the disease is also variable, but it may last four[dermatology.cdlib.org]
Myopathy
  • The patient's myopathy ceased on potassium supplementation and her other complaints resolved while on gluten-free diet. Recovery was otherwise uneventful with a rapid decline in serum CPK level.[ncbi.nlm.nih.gov]
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]
  • Abstract Itch which is one of the major, subjective symptoms in a course of bullous pemphigoid and dermatitis herpetiformis makes those two diseases totally different than other autoimmune blistering diseases.[ncbi.nlm.nih.gov]
  • 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]
  • Owing to the granular immunoglobulin (Ig) A deposition at the tips of the papillary dermis and to the subepidermal blister formation associated with neutrophilic accumulations underlying the basement membrane, DH is considered to be an autoimmune blistering[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • This condition is sometimes misdiagnosed clinically as dyshidrotic eczema, contact dermatitis, scabies, and drug eruptions.[symptoma.com]
  • We report a case of a 12-year-old girl who presented with a vesiculobullous eruption on her face, neck, trunk and genital and oral mucosa, as well as anemia, sterile pyuria, ANA (1:1280, speckled pattern) and positive anti-Sm and anti-RNP.[ncbi.nlm.nih.gov]
  • Dermatitis herpetiformis (DH) is a chronic pruritic cutaneous eruption associated with gluten-sensitive enteropathy (celiac disease [CD]) and immunoglobulin A (IgA) deposition in the skin.[ncbi.nlm.nih.gov]
Papule
  • 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]
  • We report a 7-year-old boy presenting with nonpruritic inflammatory papules on the buttocks and extensor surfaces, clinico-pathologically consistent with Sweet's neutrophilic dermatosis.[ncbi.nlm.nih.gov]
  • Dermatitis herpetiformis (DH) is an autoimmune disease that clinically manifests as pruritic vesicles and papules. The diagnosis of DH is often challenging because of its wide spectrum of clinical presentations.[ncbi.nlm.nih.gov]
  • Dermatitis herpetiformis (DH) is characterized by chronic, itching papules, seropapules, small vesicles and, exceptionally, large blisters.[ncbi.nlm.nih.gov]
  • We describe the case of a 53-year-old woman presenting papulous and papulovesicular lesions that were highly pruritic, localized mostly in the achromic areas of vitiligo and symmetrically distributed on the elbows, the buttocks, the shoulders and the[ncbi.nlm.nih.gov]
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. . Accessed April 20, 2018. McKusick VA, ed. Online Mendelian Inheritance In Man (OMIM). The Johns Hopkins University. Dermatitis Herpetiformis, Familial. Entry Number; 601230: Last Edit Date; 01/09/2006.[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]
  • He finally pronounced it as some type of eczema and sent me on my way. That was doctor number one and year number one.[realfoodallergyfree.com]
Pruritus
  • A 22 year-old Caucasian man was admitted with excoriated lesions and pruritus. Petechial lesions could be seen on volar aspect of the digits on the hands and feet.[ncbi.nlm.nih.gov]
  • Typically, young adults present with excoriations only, as the severe pruritus effectively destroys any primary lesions.[ncbi.nlm.nih.gov]
  • Treatment with dapsone led to the disappearance of cutaneous lesions and pruritus within 48 hours. Demonstration of IgA immune deposits in the dermal papillae has been the only acceptable criterion for the diagnosis of dermatitis herpetiformis.[ncbi.nlm.nih.gov]
  • Although classical DH is characterized clinically by grouped, vesicles on an erythematous base, primary lesions often are absent owing to the intense, associated pruritus.[ncbi.nlm.nih.gov]

Workup

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.

Pyuria
  • We report a case of a 12-year-old girl who presented with a vesiculobullous eruption on her face, neck, trunk and genital and oral mucosa, as well as anemia, sterile pyuria, ANA (1:1280, speckled pattern) and positive anti-Sm and anti-RNP.[ncbi.nlm.nih.gov]
Heinz Bodies
  • It progresses with erythrocytes precipitation and hemolysis characterized by Heinz bodies and "bitten cells."[dx.doi.org]

Treatment

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:

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

Prognosis

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.

Etiology

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. 

Epidemiology

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.

Sex distribution
Age distribution

Pathophysiology

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.

Prevention

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].

Summary

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. 

Patient Information

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.

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Last updated: 2019-07-11 20:35