Home

Search

Pathway AI

Account ⋅ Sign Out

Table of contents

Dermatitis herpetiformis

Key sources
The following summarized guidelines for the evaluation and management of dermatitis herpetiformis are prepared by our editorial team based on guidelines from the European Academy of Dermatology and Venereology (EADV 2021) and the European Society for the Study of Coeliac Disease (ESsCD 2019).
1
2

Guidelines

1.Screening and diagnosis

Diagnosis
As per EADV 2021 guidelines:
Diagnose dermatitis herpetiformis if both major diagnostic criteria are fulfilled - clinical manifestation compatible with dermatitis herpetiformis and positive DIF microscopy.
A
Recognize that HLA-DQ2/DQ8 positivity does not confirm the diagnosis of dermatitis herpetiformis.
B
Create free account

More topics in this section

  • Differential diagnosis

2.Diagnostic investigations

History and physical examination: elicit a medical history, perform a complete physical examination and obtain gastroenterological assessment in patients with suspected dermatitis herpetiformis.
B
Show 3 more

More topics in this section

  • Serologic testing (general principles)

  • Serologic testing (endomysial antibodies)

  • Serologic testing (tissue transglutaminase antibodies)

  • Serologic testing (epidermal transglutaminase antibodies)

  • Serologic testing (gliadin antibodies)

  • HLA genotyping

  • Baseline laboratory tests

  • Evaluation for comorbidities

3.Diagnostic procedures

Skin biopsy
As per EADV 2021 guidelines:
Perform lesional biopsy for histopathology in patients with suspected dermatitis herpetiformis.
B
Use a standardized (buffered) 4% formaldehyde (10% formalin) solution for storage and transport.
B

More topics in this section

  • DIF microscopy

  • Small bowel biopsy

4.Medical management

Dapsone
As per EADV 2021 guidelines:
Initiate dapsone in patients with dermatitis herpetiformis in the following situations:
intolerable and/or severe skin involvement
skin manifestation not responding to a correct gluten-free diet
patients not accepting or unable to adhere to a gluten-free diet
A
Do not use dapsone in patients with any of the following:
G6PD deficiency
low blood cell counts, especially anemia or neutropenia
any cardiac or pulmonary disease leading to significantly impaired tissue oxygenation
D

More topics in this section

  • Second-line therapy

  • Agents with no evidence for benefit

5.Nonpharmacologic interventions

Gluten-free diet: as per EADV 2021 guidelines, offer lifelong gluten-free diet with/without dapsone as the main therapeutic option in patients with dermatitis herpetiformis.
A
Show 4 more

More topics in this section

  • Supplements

6.Follow-up and surveillance

Follow-up: obtain the following at according intervals:
Situation
Guidance
Weekly at 1st month
History and clinical review
Every 2 weeks at 2nd and 3rd months
CBC including reticulocyte count
Methemoglobin if the daily dose of dapsone is > 150 mg weekly
Every 2 weeks at 1st 3 months
Liver function tests
Renal function tests
Every 3rd month
History and clinical review including peripheral motor neurological examination
CBC including reticulocyte count
Methemoglobin if the daily dose of dapsone is > 150 mg
Liver function tests
Renal function tests
A

7.Quality improvement

Labelling regulations: recognize the following regulations on gluten-free diets in Europe:
gluten-free labels guarantee safety for consumers with dermatitis herpetiformis and celiac disease
foods labeled as "very low gluten content" are not suitable for consumers with dermatitis herpetiformis and celiac disease
hidden gluten contamination should be avoided as it is easy to reach toxicity thresholds