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dermatitis herpetiformis - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

dermatitis herpetiformis

MONDO:0015614

Also known as:Duhring's diseaseDurhing-Brocq diseasedermatosis herpetiformis

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Overview

Dermatitis herpetiformis (DH) is a chronic autoimmune subepidermal bullous disease characterized by grouped pruritic lesions such as papules, urticarial plaques, erythema, and herpetiform vesiculae, with a predominantly symmetrical distribution on extensor surfaces of the elbows (90%), knees (30%), shoulders, buttocks, sacral region, and face of children and adults. Erosions, excoriations and hyperpigmentation usually follow. DH may also appear as a consequence of gluten intolerance.

TreatmentsNo approved treatments · 1 in pipeline

Clinical Trials2 registered · 1 recruiting

Diagnosis

Biomarker and diagnostic research for dermatitis herpetiformis has been reported in the published literature.

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

No approved treatments are currently available for dermatitis herpetiformis. An additional 1 compound holds orphan drug designation.

Orphan Drug Designations

While no drugs are FDA-approved specifically for dermatitis herpetiformis, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.

The following drugs have received orphan drug designation from the FDA for dermatitis herpetiformis. Orphan designation reflects regulatory interest and does not indicate approval for treatment.

Brand Name	Generic Name	Sponsor

Prognosis

Estimated prevalence: 1-5 in 10,000 (Uncommon).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

2 clinical trials registered, 1 recruiting. Interventions under study include other interventions and gene therapy. Research is primarily sponsored by academic and government institutions.

71 publications have been identified in PubMed for dermatitis herpetiformis. Research spans Review / Meta-Analysis (31%), Case Report / Case Series (26%), and Epidemiology / Natural History (24%).

Research Type	Count	% of Total
Research summaries	21	31%

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 1:53 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:1656

GARD

Genetic and Rare Diseases Info Center

GARD:10075

Questions for Your Care Team

→Are any of the 2 active clinical trials a good fit for my situation?
→What is the current development status of Sulfapyridine, and when might it become available?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Kisho

dermatitis herpetiformis

Overview

Diagnosis

Treatment

Orphan Drug Designations

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

Recent Publications

Latest News & Developments

Fibrillar-Type Dermatitis Herpetiformis: An Underrecognized Variant That Is Rarely Associated With Celiac Disease and Lacking Epidermal Transglutaminase Deposition: A Report of Two Cases.

dermatitis herpetiformis

Overview

Diagnosis

Treatment

Orphan Drug Designations

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

Recent Publications

Latest News & Developments

Fibrillar-Type Dermatitis Herpetiformis: An Underrecognized Variant That Is Rarely Associated With Celiac Disease and Lacking Epidermal Transglutaminase Deposition: A Report of Two Cases.