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Pemphigus erythematosus is a rare superficial pemphigus disease characterized clinically by well-demarcated, localized, erythematous, scaly, hyperkeratotic, crusted plaques, with frequent butterfly distribution over the malar area of the face (but also commonly involving trunk and scalp, and less frequently the extremities, with a photoexposed distribution). Histologically, granular deposits along the dermal-epidermal junction, in addition to intercellular deposition in the upper epidermis, are observed.
Features include very common findings: Autoimmunity, Localized skin lesion, Malar rash, and Erythematous plaque and others; and common findings: Antinuclear antibody positivity, Abnormal blistering of the skin, and Linear IgG deposits along the epidermal basement membrane zone. 14 total HPO annotations.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Skin | 7 | Localized skin lesion, Malar rash, Erythematous plaque |
Biomarker and diagnostic research for pemphigus erythematosus has been reported in the published literature.
Phenotype severity distribution: 5 very common features, 3 common features.
No clinical trials have been registered for pemphigus erythematosus.
12 publications have been identified in PubMed for pemphigus erythematosus. Research spans Case Report / Case Series (58%), Diagnostic / Biomarker (17%), and Review / Meta-Analysis (17%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 7 | 58% |
Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 1:01 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Lab test results | 2 | Antinuclear antibody positivity, Anti-neuromuscular Junction acetylcholine receptor antibody positivity |
Blood and immune system | 1 | Autoimmunity |
2 |
17% |
Research summaries | 2 | 17% |
New treatment approaches | 1 | 8% |
Zhang H (2026). [PMID: 41858604](https://pubmed.ncbi.nlm.nih.gov/41858604/). *Clin Cosmet Investig Dermatol*. [Diagnostic / Biomarker]
Hu F (2025). [PMID: 41000395](https://pubmed.ncbi.nlm.nih.gov/41000395/). *Frontiers in immunology*. [Review / Meta-Analysis]
Zheng J (2025). [PMID: 39761812](https://pubmed.ncbi.nlm.nih.gov/39761812/). *The British journal of dermatology*. [Case Report / Case Series]
Chen AW (2025). [PMID: 40059466](https://pubmed.ncbi.nlm.nih.gov/40059466/). *Acta dermato-venereologica*. [Review / Meta-Analysis]
Behera B (2025). [PMID: 40642697](https://pubmed.ncbi.nlm.nih.gov/40642697/). *Cureus*. [Case Report / Case Series]
Kirmani N (2025). [PMID: 41426874](https://pubmed.ncbi.nlm.nih.gov/41426874/). *Cureus*. [Case Report / Case Series]
Kutwin M (2025). [PMID: 39860415](https://pubmed.ncbi.nlm.nih.gov/39860415/). *Journal of clinical medicine*. [Case Report / Case Series]
Li X (2025). [PMID: 40201353](https://pubmed.ncbi.nlm.nih.gov/40201353/). *Frontiers in oncology*. [Diagnostic / Biomarker]
Gallegos Espadas D (2024). [PMID: 38817480](https://pubmed.ncbi.nlm.nih.gov/38817480/). *Cureus*. [Case Report / Case Series]
Arasiewicz H (2024). [PMID: 38784933](https://pubmed.ncbi.nlm.nih.gov/38784933/). *Postepy dermatologii i alergologii*. [Case Report / Case Series]
AI-curated news mentioning pemphigus erythematosus
Updated Sep 4, 2026
A case report highlights the successful treatment of pemphigus erythematosus using the JAK1 inhibitor upadacitinib. This study contributes to the growing body of literature on targeted therapies for autoimmune skin diseases.