Introduction

Investigating the world of dermatitis herpetiformis (DH), we uncover a persistent and intensely itchy skin condition marked by the formation of blisters. It is a manifestation of gluten-sensitive enteropathy (enteropathy implies a diseased intestinal tract), also known as celiac disease. Gluten is a protein present in wheat and various grains.¹ Dermatitis herpetiformis affects adults, with a higher prevalence among men.²   It has the potential to impact individuals of all age groups. However, it occurs in individuals who are in the age range of 30 to 40 years old.³

Signs and Symptoms of Dermatitis Herpetiformis 

The primary indication experienced by individuals with dermatitis herpetiformis is itching.⁴

• Patients commonly describe a rash that typically appears on the outer surfaces of the elbows, knees, buttocks, and scalp. 
• Additionally, there may be accompanying symptoms related to associated conditions such as gluten-sensitive enteropathy (GSE), such as bloating, diarrhea, and other gastrointestinal issues. 
• Patients may also exhibit symptoms of other autoimmune diseases, including hypothyroidism.⁴
• Before the formation of bruises, individuals may experience a burning sensation. By the time a patient seeks a physical examination, the bruises are typically scratched off, resulting in the rash appearing as erosions.
• Dermatitis herpetiformis can impact the condition of your teeth. It may lead to the appearance of pits, discoloration, or horizontal grooves on the tooth enamel. Further, you may also experience the occurrence of canker sores (small wounds in the mouth or on the gums).^2,3

When to see a doctor

It is crucial to seek medical attention promptly when you start experiencing symptoms of dermatitis herpetiformis. 

• The itchiness and a burning sensation, which can significantly impact your daily life and well-being. 
• Delaying seeking medical help may prolong your symptoms and hinder your quality of life, so do not hesitate to reach out to your healthcare provider for timely assistance
• Visible bumps and blisters from dermatitis herpetiformis can make social situations uncomfortable, and the persistent itchiness can be distracting and disrupt your sleep. It is important to follow your healthcare provider’s treatment instructions for relief
• A skin specialist, known as a dermatologist, can assist you in managing and treating skin-related symptoms. 
• If you have celiac disease and need to follow a gluten-free diet, it might be beneficial to consult a dietician or nutritionist who can provide guidance on suitable dietary choices.³

Causes of dermatitis herpetiformis

Dermatitis herpetiformis can be caused by several factors, including the following: 

• Gluten diet: Consuming food items containing gluten which triggers your immune system, leading to the production of antibodies (IgA). These antibodies are then deposited in the skin, resulting in the formation of itchy bumps and blisters.³
• Gluten-containing foods should be avoided (Barley, Bread (made from rye and wheat), Cakes, Crackers, Cereals, Pasta).³
• Genetics: Several studies have established a strong association between dermatitis herpetiformis and specific HLA types.⁴ HLA-DQ2 and HLA-DQ8 genes are linked with dermatitis herpetiformis.³
• Environmental factors: The main environmental factor implicated in the development of dermatitis herpetiformis is the consumption of dietary gluten, specifically gliadin, which is a component of gluten. Other environmental factors that can contribute to dermatitis herpetiformis include exposure to iodine, which can trigger disease flares.⁴

Risk factors for dermatitis herpetiformis

Dermatitis herpetiformis is more commonly observed in individuals with a northern European background.¹

The presence of certain diseases can heighten the risk of developing dermatitis herpetiformis, including 

• Autoimmune thyroid disease
• Type 1 diabetes
• Celiac disease
• Sjogren’s syndrome (a chronic autoimmune condition that diminishes the production of moisture by glands in the eyes and mouth, a condition that persists throughout one’s life.)⁵
• Lupus.¹

Individuals who have a diet full of gluten are also at higher risk of developing dermatitis herpetiformis. Food items like bread, pasta, cakes, and barley are inclusive of gluten. One should avoid gluten-containing food if they are susceptible to this condition.³

Diagnosis of dermatitis herpetiformis

To diagnose dermatitis herpetiformis, a medical professional will conduct a physical examination of your skin. Additional tests may be conducted to confirm the diagnosis, including:

• Skin biopsy: Your doctor might collect a skin sample to evaluate the indications of dermatitis herpetiformis.³
• Blood test: There are two antibodies, anti-endomysial and anti-tissue transglutaminase, commonly found in individuals with celiac disease. If your antibody tests show positive results and your skin biopsy confirms the presence of dermatitis herpetiformis, you have celiac disease. In some cases, an intestinal biopsy may be necessary to confirm the presence of celiac disease.³

These tests are essential in determining whether you have dermatitis herpetiformis or another skin condition.³

Treatment of dermatitis herpetiformis

The treatment options for dermatitis herpetiformis consist of:

• Following a gluten-free diet.³
• A gluten-free diet aids in the management of dermatitis herpetiformis through the following benefits: 
• Reducing dependency on medications to control the skin condition.
• Decreasing the likelihood of developing additional autoimmune diseases.
• Lowering the risk of intestinal lymph cancer.
• Improving the state of any gluten-sensitive intestinal disease.
• Enhancing nutrition and promoting healthy bone density.
• Taking an oral antibiotic prescribed by your doctor. It can provide relief from itching within an hour, often even before 48 hours. It may be necessary to continue taking prescribed antibiotics for one to two years to prevent the formation of additional bumps and blisters associated with dermatitis herpetiformis.³

Prevention & management of dermatitis herpetiformis

Currently, there are no known methods for preventing dermatitis herpetiformis. However, adopting a gluten-free diet can help lessen the chances of experiencing symptom flare-ups.

Management of dermatitis herpetiformis: The management of dermatitis herpetiformis requires a multidisciplinary team comprising a dermatologist, dietitian, gastroenterologist, nurse practitioner, and neurologist.⁴

• The primary strategy for long-term management of dermatitis herpetiformis is strict adherence to a gluten-free diet, which not only improves skin changes over time but is also crucial for managing associated gluten-sensitive enteropathy (GSE; enteropathy implies diseased intestinal tract). 
• Collaborating with an experienced dietitian is recommended for optimal support as maintaining a strict gluten-free diet can be challenging.
• Due to the slow response of the skin condition to a gluten-free diet, most dermatitis herpetiformis patients require the use of medication for short to medium-term control of the disease.⁴

Complications of dermatitis herpetiformis

Dermatitis herpetiformis is not just a skin condition. It is connected to a condition called gluten-sensitive enteropathy (GSE), which affects the intestinal tract. 

• Gluten-sensitive enteropathy (GSE) can result in difficulties absorbing nutrients from food, leading to:
  • Anaemia
  • Weight loss
  • Weak bones (osteoporosis)
  • In children, GSE can impact growth and contribute to a shorter height.
• Although rare, dermatitis herpetiformis has been occasionally associated with neurological disorders such as:
  • Ataxia (loss of muscle control)
  • Neuropathy (nerve damage)

It is crucial to be aware of these potential complications and consult with your healthcare provider for appropriate management. ⁴ 

Research facts about dermatitis herpetiformis

• Each year, 0.4 to 2.6 out of every 100,000 individuals in the United States are diagnosed with dermatitis herpetiformis.³
• Symptoms of celiac disease are present in fewer than 20 percent of individuals with dermatitis herpetiformis.²
• Several studies indicate that dermatitis herpetiformis is more prevalent in males compared to females, with a male-to-female ratio ranging from 1.5 to 2:1.⁴

FAQ’s

Conclusion

Dermatitis herpetiformis is a long-lasting condition that involves autoimmune reactions and results in a blistering rash. This rash can be intensely itchy and affects the outer surfaces of the body. It is important to exercise caution and seek appropriate medical attention for proper diagnosis and management of this condition.⁴

Disclaimer: The information included on this site is for educational purposes only and is not intended to be a substitute for medical treatment by a healthcare professional. Because of unique individual needs, the reader should consult their physician to determine the appropriateness of the information for the reader’s situation. 

Reference

1. Dermatitis Herpetiformis | Cedars-Sinai [Internet]. Available from: https://www.cedars-sinai.org/health-library/diseases-and-conditions/d/dermatitis-herpetiformis.html

2. Dermatitis Herpetiformis – NIDDK [Internet]. Available from: https://www.niddk.nih.gov/health-information/professionals/clinical-tools-patient-management/digestive-diseases/dermatitis-herpetiformis

3. Dermatitis Herpetiformis: Celiac Disease, Symptoms & Treatment [Internet]. Available from: https://my.clevelandclinic.org/health/diseases/21460-dermatitis-herpetiformis

4. Dermatitis Herpetiformis – StatPearls – NCBI Bookshelf [Internet]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK493163/

5. Sjögren’s Syndrome: Causes, Symptoms, Diagnosis & Treatments [Internet]. Available from: https://my.clevelandclinic.org/health/diseases/4929-sjogrens-syndrome