Dermatitis herpetiformis

Summary about Disease


Dermatitis herpetiformis (DH) is a chronic autoimmune blistering skin condition strongly associated with celiac disease. It's characterized by intensely itchy papules and vesicles (small blisters) that typically appear symmetrically on the elbows, knees, buttocks, and scalp. The underlying cause is a reaction to gluten, a protein found in wheat, barley, and rye.

Symptoms


Intensely itchy, small blisters (vesicles) and bumps (papules).

Symmetrical distribution on the body, commonly affecting elbows, knees, buttocks, scalp, and back.

Scratching often breaks the blisters, leading to crusts and excoriations.

The itch is often described as burning, stinging, or prickling.

Hyperpigmentation or hypopigmentation (darkening or lightening of the skin) may occur after lesions heal.

Causes


Dermatitis herpetiformis is caused by an autoimmune reaction triggered by gluten ingestion in genetically susceptible individuals.

The immune system produces antibodies (specifically IgA) that are deposited in the skin, leading to inflammation and blister formation.

It is strongly associated with celiac disease, although many individuals with DH may not have classic gastrointestinal symptoms of celiac disease.

Genetic factors play a significant role, with a strong association to the HLA-DQ2 and HLA-DQ8 genes.

Medicine Used


Dapsone: This is the primary medication used to control the skin symptoms of DH. It is not a cure but can significantly reduce itching and blister formation.

Sulfapyridine: An alternative to dapsone for individuals who cannot tolerate dapsone.

Gluten-free diet: This is the most important long-term treatment. Strictly adhering to a gluten-free diet can reduce or eliminate the need for medication.

Is Communicable


No, dermatitis herpetiformis is not contagious. It is an autoimmune condition and cannot be spread from person to person.

Precautions


Strict gluten-free diet: This is the most critical precaution. Read food labels carefully and avoid cross-contamination.

Avoid scratching: Scratching can worsen the lesions and increase the risk of infection.

Gentle skin care: Use mild, fragrance-free soaps and moisturizers.

Sun protection: Protect affected areas from sun exposure.

Regular medical follow-up: Monitor the condition and adjust medication as needed.

How long does an outbreak last?


Without treatment (dapsone or gluten-free diet), outbreaks can be chronic and persistent, lasting for weeks, months, or even years. With dapsone, symptoms can be controlled quickly, often within a few days. The length of time to remission while maintaining a strict gluten-free diet varies from person to person and can take months to years.

How is it diagnosed?


Skin biopsy: A small sample of skin is taken from around a blister and examined under a microscope. The presence of IgA deposits in a granular pattern in the dermal papillae is characteristic of DH.

Direct immunofluorescence (DIF): This test identifies the IgA deposits in the skin biopsy sample.

Blood tests: To check for antibodies related to celiac disease (e.g., anti-tissue transglutaminase (tTG) and anti-endomysial (EMA) antibodies).

Endoscopy and intestinal biopsy: May be performed to assess for celiac disease, especially if blood tests are positive or if gastrointestinal symptoms are present.

Timeline of Symptoms


The onset of symptoms can vary. It may start with intense itching, followed by the appearance of small blisters and bumps over days or weeks. The lesions typically appear in crops, with new lesions developing while older ones are healing. Without treatment, this cycle of itching, blistering, and healing can continue indefinitely.

Important Considerations


Dermatitis herpetiformis is a lifelong condition that requires ongoing management.

Adhering to a strict gluten-free diet is crucial for long-term control and may reduce the need for medication.

Even small amounts of gluten can trigger symptoms.

Regular monitoring by a dermatologist and gastroenterologist is recommended.

DH is associated with an increased risk of other autoimmune conditions, so it's important to be aware of potential symptoms.

Early diagnosis and treatment can improve quality of life and reduce the risk of complications.