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

Rare Disease

dermatitis herpetiformis, familial

MONDO:0011024

Also known as:hereditary dermatitis herpetiformisBrocq-Duhring diseaseDH

Find Clinical TrialsTrials

Overview

Dermatitis herpetiformis is a rare, chronic, skin disorder characterized by groups of severely itchy blisters and raised skin lesions. These are more common on the knees, elbows, buttocks and shoulder blades. The slow onset of symptoms usually begins during adulthood, but children can also be affected. Other symptoms mayinclude fluid-filled sores; red lesions that resemble hives; and itchiness, rednessand burning. The exact cause of this disease is not known,but it is frequently associated with the inability to digest gluten. People with this disease are typically treated with the drug dapsone.

GeneticsAutosomal dominant

Clinical Trials2 registered · 1 recruiting

ResearchLimited published research (4 articles)

Clinical Presentation

Features include: Pruritus.

Organ System	Phenotype Count	Example Features
Skin	1	Pruritus

Explore clinical features

Diagnosis

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

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.

5 publications have been identified in PubMed for dermatitis herpetiformis, familial. Research spans Diagnostic / Biomarker (25%), Review / Meta-Analysis (25%), and Basic Science / Preclinical (25%).

Recent Publications

Medina Bethencourt M (2026). [PMID: 40995939](https://pubmed.ncbi.nlm.nih.gov/40995939/). *Clin Pediatr (Phila)*. [Diagnostic / Biomarker]
Simionescu O (2025). [PMID: 40098967](https://pubmed.ncbi.nlm.nih.gov/40098967/). *Front Immunol*. [Review / Meta-Analysis]
Su Y (2025). [PMID: 41393268](https://pubmed.ncbi.nlm.nih.gov/41393268/). *Clin Cosmet Investig Dermatol*. [Basic Science / Preclinical]
Kotze LMDS (2025). [PMID: 40699045](https://pubmed.ncbi.nlm.nih.gov/40699045/). *Arq Gastroenterol*. [Epidemiology / Natural History]

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 12:51 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:601230

GARD

Genetic and Rare Diseases Info Center

GARD:1917

Questions for Your Care Team

→Are any of the 2 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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Kisho

dermatitis herpetiformis, familial

Overview

Clinical Presentation

Explore clinical features

Diagnosis

Research Landscape

Recent Publications

Medical References

External Resources

OMIM

GARD

Questions for Your Care Team

Questions for Your Care Team

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, familial

Overview

Clinical Presentation

Explore clinical features

Diagnosis

Research Landscape

Recent Publications

Medical References

External Resources

OMIM

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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.