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Dermatitis herpetiformis: the skin as a sign of coeliac disease

Dermatitis herpetiformis is the skin manifestation of coeliac disease. Find out about its symptoms, diagnosis, treatment and link to gluten.

Dermatitis herpetiformis: the skin as a key to diagnosing coeliac disease

Coeliac disease does not always present with clear digestive symptoms. In some patients, the clinical manifestations are varied or seemingly unrelated, and reaching a diagnosis can be complex. In this context, the skin can become a key factor.

Dermatitis herpetiformis is a skin manifestation of coeliac disease and, in some cases, is the sign that allows us to identify previously undiagnosed gluten-sensitive enteropathy.

Recognising its characteristic lesions is important: many patients have few or no digestive symptoms, which contributes to underdiagnosis and delays the start of an appropriate gluten-free diet.

What is dermatitis herpetiformis?

Dermatitis herpetiformis (DH), also known as Duhring’s dermatitis, is an autoimmune blistering disease associated with gluten sensitivity.

Although it is a rare condition, over 90% of patients have some degree of gluten-sensitive enteropathy, which can range from intraepithelial lymphocytosis to advanced villous atrophy.

However, only a minority develop the classic digestive symptoms of coeliac disease, which explains why many cases go undetected for years.

A person adjusts their clothes, scratching an itch on their forearm while wearing a fitted white long-sleeve shirt

RThe relationship between dermatitis herpetiformis and coeliac disease

The relationship between dermatitis herpetiformis and coeliac disease is very close. Both the skin lesions and the intestinal involvement improve when gluten is removed from the diet and reappear when it is reintroduced.

For this reason, dermatitis herpetiformis is now considered a form of coeliac disease with a predominantly cutaneous manifestation.

Causes of dermatitis herpetiformis: why does it occur?

The aetiopathogenesis of dermatitis herpetiformis is complex and multifactorial. Genetic, immunological and environmental factors are involved in its development, with gluten being the main trigger.

The role of gluten in dermatitis herpetiformis

In susceptible individuals, the ingestion of gluten triggers an abnormal immune response that promotes the formation of IgA deposits in the skin.

These deposits accumulate mainly in the dermal papillae and trigger the inflammation responsible for the skin lesions and the intense itching typical of the condition.

Genetic predisposition: HLA DQ2 and DQ8

As with coeliac disease, there is a clear genetic predisposition. Most patients with dermatitis herpetiformis carry the HLA DQ2 haplotypes and, to a lesser extent, HLA DQ8.

These genetic markers are present in a very high percentage of patients with coeliac disease and reflect the shared immunogenetic basis between the two conditions.

⚫ Symptoms of dermatitis herpetiformis

Recurrent outbreaks of skin lesions causing intense itching are the main symptoms associated with dermatitis herpetiformis. Let’s look at this in more detail:

⚫ Diagnosis of dermatitis herpetiformis

The diagnosis of dermatitis herpetiformis combines clinical suspicion with specific histological and immunological tests:

⚫ Treatment of dermatitis herpetiformis

For the proper treatment of dermatitis herpetiformis, it is necessary to address both the skin involvement and the underlying coeliac disease. This treatment consists of:

Why does dermatitis herpetiformis often go undiagnosed?

Dermatitis herpetiformis appears to be underdiagnosed, but why? There are several reasons for this:

  • One of the main reasons is that the lesions can be mistaken for other, more common skin conditions. Furthermore, if the patient scratches vigorously, the initial clinical appearance of the lesions is likely to change.
  • Furthermore, as this is a condition that occurs in flare-ups, some patients seek medical advice only after the typical lesions have already partially disappeared.
  • Added to this is the fact that most patients do not present clear digestive symptoms, making it difficult to link the skin manifestations to underlying coeliac disease.

The importance of early diagnosis

In any case, identifying dermatitis herpetiformis in good time is essential. Not only to alleviate the itching and skin lesions (and, consequently, improve the patient’s quality of life), but also to diagnose coeliac disease, which, in many cases, remains undiagnosed.

Early diagnosis allows:

  1. Starting an appropriate gluten-free diet
  2. Avoid complications related to coeliac disease
  3. Reduce unnecessary treatments
  4. Detect other associated autoimmune diseases
  5. Identify possible undiagnosed cases within the family

Other clues that may indicate coeliac disease

Note! Dermatitis herpetiformis is not the only extragastrointestinal manifestation that may point to coeliac disease.

In some patients, other signs may be present which, although they may seem unrelated, can also form part of the same process. For example, iron-deficiency anaemia, recurrent mouth ulcers or changes in tooth enamel.

Therefore, the diagnosis of coeliac disease requires a holistic view of the patient and the ability to link symptoms from different organs and systems.

Frequently asked questions about dermatitis herpetiformis

Conclusion: the importance of recognising the skin manifestations of coeliac disease

The skin can sometimes be the key to diagnosing coeliac disease that has gone unnoticed for years.

Recognising dermatitis herpetiformis and other extra-digestive manifestations allows us to identify patients with subclinical coeliac disease and initiate an appropriate gluten-free diet at an early stage.

Therefore, healthcare professionals must maintain a high level of clinical suspicion and learn to link seemingly isolated symptoms that are, in fact, part of the same process.

Paloma Borregón Nofuentes

  • Specialist in Dermatology
  • PhD cum laude with the thesis “Dermatitis herpetiformis as a manifestation of coeliac disease”
  • Member of the scientific committee of the Spanish Federation of Coeliac Associations (FACE)
  • Head of the Beteré-Marcos Clinical Dermatology Unit (Madrid)
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