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Dermatitis herpetiformis
Published: September 18, 2026Updated: September 18, 2026
Important: Informational content intended for healthcare professionals. It does not replace clinical evaluation or establish a doctor-patient relationship.
Overview
- Definition and synonyms
- Dermatitis herpetiformis (DH) : a chronic, pruritic, autoimmune bullous dermatosis characterized by intensely pruritic papules and vesicles on extensor surfaces, almost invariably associated with gluten-sensitive enteropathy (in practice, a cutaneous manifestation of celiac disease). Salmi 2020 .
- It is currently considered a cutaneous manifestation of celiac disease, sharing a genetic basis HLA-DQ2/DQ8, IgA autoantibodies against tissue transglutaminase (TG2) and against epidermal transglutaminase (TG3). Collin 2003 , Ingen-Housz-Oro 2011 .
- It is not related to herpes viruses ; “herpetiform” refers to the clustered pattern of vesicles. Ingen-Housz-Oro 2011 .
- Synonyms: Duhring’s disease, Duhring-Brocq disease, morbus Duhring. Bogenrieder 2003 .
- History: First described in 1884 by Louis Adolphus Duhring , a Philadelphia dermatologist; the modern historical review details his biography and the original description. Bogenrieder 2003 .
- Epidemiology
- Rare disease but with wide geographical variation:
- Prevalence : ~10–75/100,000 in series from Northern Europe and Finland; Finland reported 75/100,000 in 2009. Salmi 2011 , DermNet NZ .
- Incidence : 0.98–5.2/100,000/year according to series from Utah (USA) and Finland. Smith 1992 , Salmi 2011 .
- More frequent in individuals of northern European descent; rare in Afro-descendant and Asian populations, although series from India demonstrate that it is also present in non-Western countries. Handa 2018 .
- Peak age of onset: 30–60 years; pediatric cases exist but are less frequent. Ingen-Housz-Oro 2011 , Salmi 2011 .
- Sex ratio : slight male predominance (≈1.1–1.5:1). Salmi 2011 , Smith 1992 .
- DH is much less common than celiac disease (approximately 1 case of DH for every 8 cases of celiac disease in Finland). Salmi 2011 .
- Rare disease but with wide geographical variation:
- ICD Codes
- ICD-10 : L13.0 – Dermatitis herpetiformis.
- ICD-11 : EB44 – Dermatitis herpetiformis.
- Diagnostic checklist (quick)
- Essential clinic
- Intense, chronic itching.
- Papules/vesicles grouped on extensor surfaces (elbows, knees), buttocks and lumbosacral region.
- History compatible with unrestricted gluten intake.
- Laboratory / minimal cabinet
- total serum IgA.
- IgA anti-TG2 ± IgA anti-endomysium (or specific IgG if IgA deficiency).
- Blood count, liver profile, creatinine, G6PD (if dapsone use is being considered).
- Biopsy
- Active lesion punch for HE (neutrophilic subepidermal bullous dermatosis).
- Punch of intact perilesional skin for IFD (granular IgA in dermal papillae).
- Confirmation
- Diagnosis of DH is highly probable if:
- Typical clinical presentation + positive IFD.
- Additional support: positive celiac serology and/or duodenal enteropathy.
- Diagnosis of DH is highly probable if:
- Essential clinic
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