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Dermatitis Herpetiformis

What is Dermatitis Herpetiformis?

Dermatitis herpetiformis, otherwise known as Brocq- Duhring disease refers to a persistent blistering dermatological problem described by clustered excoriations, urticaria and vesicles that contain watery fluid located characteristically on the elbows, knees, back and buttocks.

Several people label it as gluten rash. Its subepidermal bullous presentation is due to an individual’s atypical immunologic reaction involving the IgA antibodies. Here, an individual has developed intolerance to a specific protein present in wheat, barley and rye which is known as gluten.

Fluid accumulation, vascular dilatation and cellular infiltration come about within the dermis. In addition, this intolerance results to a disorder affecting the alimentary canal, termed celiac disease, which is directly associated to dermatitis herpetiformis.

Regardless of its term, this condition is not in any relation to herpes virus. However, the clinical presentation in the skin may have a form comparable to herpes.

The said condition is named after an American doctor and professor of dermatology from the University of Pennsylvania, Dr. Louis Adolphus Duhring who first reported the disease in year 1884. The next report involving the condition was made in 1967, pointing out the association between dermatitis herpetiformis and gluten intolerance, even if the exact pathophysiology is undefined.

Approximations of the global prevalence of the disease range from 1 out of 400 or 1 in every 10,000 population. While dermatitis herpetiformis also affects children and adults, commonly affected are individuals aged 15 to 40 years old. 2/3 of the reported cases are men. Around 80% of the affected people have gluten enteropathy.

The condition is diagnosed by obtaining a skin biopsy of the upper papillary dermis and directly subjecting the sample to immunofluorescence.

Symptoms

Dermatitis herpetiformis is clinically presented with extremely itchy, persistent flesh-colored to reddish papulovesicular lesions which are commonly scattered symmetrically on the body and are mostly found in the scalp, shoulder, elbows, back, knees and buttocks. Primarily, patient reports itching and burning which arise twelve hours prior to the appearance of the gluten rash. The uncomfortable feeling continues up to ten days.
Initially, patient may exhibit detectable indicators of dermatitis herpetiformis within the dermis. These are in the form of papules and vesicles which appear originally on the face, shoulder, inside the mouth and inferior point of the spinal column.
The papules and vesicles may range in size from very insignificant up to one centimeter in diameter.
The bumps are intensively itchy, with a tremendous desire to scratch them off resulting to skin erosion and crusting. At times, even before the appearance of the blisters on the skin, patient may already complain of a severe itching and burning feeling.
When left untreated, the condition may become severe, which may be dependent on the individual’s level of gluten intake.
The rash undergoes three phases. During the first stage, a slight pigmentation on the skin can be observed where the lesions emerge. In the second phase, the cutaneous bumps become apparent papulovesicular lesions which tend to crop up in clusters. The third stage is lesional healing. Older bumps leave after some time and finally cause changes in skin color, which can be either hyperpigmented or hypopigmented.
While the disorder is labeled as a lifetime condition, intervals of exacerbation and remission are usual.

Causes

The disease is idiopathic; however it is radically linked to gluten sensitivity and immunoregulatory disturbance. The leading assumption to explain the disease occurrence includes genetic participation together with diet rich in gluten. It may arise once the patient is subjected to chronic stimulation of the intestinal mucosa by ingested gluten-rich foods. Barley, wheat, oats and rye all contain a protein called gluten.

Rash appears when protein gluten adheres with IgA, an antibody formed in the intestinal lining. After they penetrate the circulation, they obstruct the tiny blood vessels in the cutaneous layer. Their deposition in the papillary dermis activates an immunologic cascade. Neutrophils are drawn towards the clogged up area and liberate a potent chemical known as complements, which cause the appearance of rash.

There are many investigations which have identified several prospective factors which participate in the development of Brocq- Duhring disease. Immunological researches have confirmed results comparable to celiac disease especially on the involved autoantigen. In this condition, a cystosolic enzyme known as epidermal transglutaminase is involved.

Treatment

Dermatitis herpetiformis can be successfully managed by correct medications alone. The disease responds favorably to Dapsone. Daily dose requirement may range from 50 to 300 milligrams. Classified as an antibacterial agent, its purpose in the management of the condition which isn’t bacterial in origin is poorly determined. It is assumed to have some anti-inflammatory effects.

For majority of most individuals, this medication is an effective treatment that will rapidly improve the disease in just a few days. It responds so quickly that itching is radically lessened in just two to three days. In spite of this, as soon as the damage has extended to the gastrointestinal system, as in the case of celiac disease, this treatment modality has already no effect. Continued use can result to adverse reaction involving the blood such as anemia; therefore, blood must be regularly examined for the first 3 months.

Ultrapotent topical steroids, systemic corticosteroid creams and Sulfapyridine tablets are alternative yet lesser effective pharmacological modality intended for patients who cannot tolerate or are hypersensitive to the drug of choice.

Diet

To facilitate disease control, a strict gluten-free diet should be observed as lifetime requirement. This modification can drastically decrease related intestinal damage and other complications. People with DH must avoid consuming foods which contain gluten such as rye, barley, oats and wheat. When a patient has already been under gluten-free diet plan for quite some time, typically, the doctor either reduces the dosage of Dapsone or discontinue.

For restrictive dietary compliance, doctors lay emphasis on the significance of educating, motivating and supporting the affected individuals, as well as referring them to a dietician/nutritionist and support systems. The points enumerated below are rationales why a rigorous gluten-free diet is solidly suggested.

This will decrease the patient’s Dapsone dosage, thus decreasing also the patient’s risk for adverse effects concerning the blood.
Patients with related gluten enteropathy will improve.
They will have enriched nourishment and improved bone density.
Strict compliance can diminish the person’s chance of developing and autoimmune diseases and most likely, intestinal lymphoma.

With proper treatment, the prognosis is very good. Even if it takes probably months to years for diet modification to improve dermatitis herpetiformis, if maintained in addition to medications, clinical features can be lowered gradually and may totally cure the condition. The bowel mucosa, cutaneous appearance and concentration of antibodies circulating in the bloodstream will normalize.