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Pemphigus erythematosus
Other names	Senear–Usher syndrome
Photo of a patient with pemphigus erythematosus showing crusted blisters
Pronunciation	/ˈpɛmfɪɡəs ˌɛrɪθɪməˈtoʊsəs/
Specialty	Dermatology
Symptoms	Flaccid bullae (blisters), crusted plaques, photosensitivity
Usual onset	40 - 60 years of age
Duration	Long term
Causes	Unclear
Diagnostic method	Based on symptoms, blood tests, and skin biopsy
Differential diagnosis	Pemphigus foliaceus, systemic lupus erythematosus
Treatment	Corticosteroids, mycophenolate mofetil, and anti-CD20 antibodies

Pemphigus erythematosus (Senear-Usher Syndrome) is a rare form of pemphigus with features of pemphigus foliaceus and lupus erythematosus. It was first described by Francis Senear and Barney Usher at the University of Illinois College of Medicine in 1926.

Signs and symptoms

Patients with pemphigus erythematosus typically present with superficially eroded lesions, or vesiculobullae, that may ooze and crust. This is especially common in areas of the body that are exposed to the sun, like the back, upper chest, and face.

The symptoms of pemphigus erythematosus usually appear slowly and progress slowly. The patient might not even be aware that their pemphigus erythematosus is photosensitive, even though the distribution of the lesions should indicate that they were induced by sunlight.

Causes

Pemphigus patients experience an autoimmune reaction that targets desmosomes. pemphigus erythematosus relapse has been linked to atorvastatin use. There has been one report of a new case of pemphigus erythematosus following topical ingenol mebutate treatment.

Diagnosis

Skin biopsies are used to diagnose pemphigus erythematosus. Immunofluorescence studies are performed on perilesional skin.

Treatment

It has been demonstrated that immunoadsorption and plasmapheresis are useful treatments for pemphigus erythematosus.

See also

• Pemphigus
• List of cutaneous conditions
• List of conditions caused by problems with junctional proteins

References

1. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
2. Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). Page 562. McGraw-Hill. ISBN 0-07-138076-0.
3. Steffen, Charles; Thomas, David (October 2003). "The Men Behind the Eponym: Francis E. Senear, Barney Usher, and the Senear-Usher Syndrome". The American Journal of Dermatopathology. 25 (5): 432–436. doi:10.1097/00000372-200310000-00011. ISSN 0193-1091. PMID 14501294.
4. ^ Bharti, Rakesh (December 6, 2022). "Pemphigus Erythematosus: Practice Essentials, Etiology, Epidemiology". Medscape Reference. Retrieved February 4, 2024.
5. Oktarina, Dyah A. M.; Poot, Angelique M.; Kramer, Duco; Diercks, Gilles F. H.; Jonkman, Marcel F.; Pas, Hendri H. (October 1, 2012). "The IgG "Lupus-Band" Deposition Pattern of Pemphigus Erythematosus". Archives of Dermatology. 148 (10). American Medical Association (AMA): 1173–1178. doi:10.1001/archdermatol.2012.1896. ISSN 0003-987X. PMID 22801864.
6. Lo Schiavo, Ada; Puca, Rosa Valentina; Romano, Francesca; Cozzi, Roberto (2014). "Pemphigus erythematosus relapse associated with atorvastatin intake". Drug Design, Development and Therapy. 8. Informa UK Limited: 1463–1465. doi:10.2147/dddt.s66666. ISSN 1177-8881. PMC 4173814. PMID 25258514.
7. Kim, YoungJae; Yang, HeeJoo; Lee, WooJin; Won, ChongHyun; Chang, SungEun; Lee, MiWoo; Choi, JeeHo (2021). "A case of new-onset pemphigus erythematosus after topical application of ingenol mebutate". Indian Journal of Dermatology. 66 (5). Medknow: 563–565. doi:10.4103/ijd.ijd_820_20. ISSN 0019-5154. PMC 8751704. PMID 35068520.
8. Ogata, Kimiko; Yasuda, Kayo; Matsushita, Mitsuji; Kodama, Hajime (1999). "Successful Treatment of Adolescent Pemphigus Vulgaris by Immunoadsorption Method". The Journal of Dermatology. 26 (4). Wiley: 236–239. doi:10.1111/j.1346-8138.1999.tb03463.x. ISSN 0385-2407. PMID 10343469. S2CID 22757265.
9. Egan, Conleth A.; Meadows, Kappa P.; Zone, John J. (2000). "Plasmapheresis as a steroid saving procedure in bullous pemphigoid". International Journal of Dermatology. 39 (3). Wiley: 230–235. doi:10.1046/j.1365-4362.2000.00876.x. ISSN 0011-9059. PMID 10759969. S2CID 23582853.

Further reading

• Desai, Anand; Harris, Jessica; Motaparthi, Kiran (2018). "Enzyme-linked immunosorbent assay as a helpful diagnostic tool for pemphigus erythematosus with equivocal histologic and immunofluorescent findings". Dermatology Online Journal. 24 (8). doi:10.5070/D3248041130. ISSN 1087-2108. PMID 30677852.
• Amerian, Mary L.; Ahmed, A. Razzaque (1984). "Pemphigus erythematosus". Journal of the American Academy of Dermatology. 10 (2). Elsevier BV: 215–222. doi:10.1016/s0190-9622(84)70025-9. ISSN 0190-9622.

External links

• Dermatology Advisor
• VisualDx

• Chronic blistering cutaneous conditions
• Syndromes