Pemphigus herpetiformis is a cutaneous condition, a clinical variant of pemphigus that combines the clinical features of dermatitis herpetiformis with the immunopathologic features of pemphigus.[1]
| Pemphigus herpetiformis | |
|---|---|
| Other names | Acantholytic herpetiform dermatitis, Herpetiform pemphigus, Mixed bullous disease, Pemphigus controlled by sulfapyridine |
| Specialty | Dermatology |
Pathophysiology
editPemphigus Herpetiformis is an IGg mediated autoantibodies that affect the epidermal layer of the skin.[2]
Diagnosis
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Diagnosis of pemphigus herpetiformis is usually based on a combination of clinical appearance, skin biopsy, direct immunofluorescence, and serologic testing for pemphigus-related autoantibodies.[3] Clinically, the condition may present with red, itchy erosions and blisters arranged in a herpetiform pattern, and it is often described as milder than classic pemphigus vulgaris or pemphigus foliaceus.[3] Histological findings may include subcorneal pustules and eosinophilic spongiosis, while acantholysis may be minimal or absent compared with other forms of pemphigus.[4] Because its symptoms can resemble dermatitis herpetiformis, linear IgA bullous dermatosis, bullous pemphigoid, and other autoimmune blistering diseases, diagnosis requires careful comparison of clinical, histological, and immunofluorescence findings.[5]
The autoimmune findings are usually associated with IgG antibodies against desmoglein 1, although some cases involve antibodies against desmoglein 3 or other desmosomal proteins.[6] Reviews of reported cases describe pemphigus herpetiformis as a rare disorder with variable clinical, histologic, immunopathologic, and treatment features.[5] Treatment approaches reported in the literature include systemic corticosteroids, dapsone, and other immunosuppressive or steroid-sparing therapies, depending on disease severity and patient response.[7] Because pemphigus herpetiformis is rare and can overlap with other blistering disorders, clearer public information may help reduce confusion, delayed recognition, and mismedicine-related diagnostic errors.[8]
See also
editReferences
edit- ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
- ↑ Sandhu, Neil (2019-01-09). "Pemphigus Herpetiformis: Background, Pathophysiology, Epidemiology". Neil Sandhu, MD Dermatologist (Medical/Cosmetics) and Mohs Surgeon, Gulf Coast Dermatology.
{{cite journal}}: Cite journal requires|journal=(help) - 1 2 Khan Mohammad Beigi, Pooya (2018). A Clinician's Guide to Pemphigus Vulgaris. Springer International Publishing. doi:10.1007/978-3-319-67759-0. ISBN 978-3-319-67758-3.
- ↑ Syed, H. A. (2025). Pemphigus Herpetiformis. StatPearls Publishing. PMID 29494022.
- 1 2 Costa, L. M. C.; Cappel, M. A.; Keeling, J. H. (2019). "Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria". International Journal of Dermatology. 58 (9): 997–1007. doi:10.1111/ijd.14395. PMID 30900757.
- ↑ On, H. R.; Hashimoto, T. (2015). "Pemphigus herpetiformis with IgG autoantibodies to desmoglein 1 and desmocollin 1". British Journal of Dermatology. 172 (4): 1144–1146. doi:10.1111/bjd.13419. PMID 25244249.
- ↑ Alkeraye, S.; AlZamil, L. R.; Alenazi, S. (2020). "Dapsone in the Management of Pemphigus and Pemphigoid: Rediscovery of its Long-Lost Efficacy". Cureus. 12 (6) e8805. doi:10.7759/cureus.8805. PMC 7381850. PMID 32724751.
- ↑ Mousapour, Niki (2026). "Diagnostic Errors as a Form of Mismedicine". Misdiagnosis Association and Research Institute. Retrieved 2026-05-12.
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