Scalp Involvement In Pemphigus: A Retrospective Cohort Study Of Clinical And Immunopathologic Features
Keywords:
Autoimmune blistering diseases , Pemphigus, Desmoglein 1, Rituximab, scalp, alopeciaAbstract
Introduction: Pemphigus is a rare autoimmune blistering disease characterized by the presence of autoantibodies against desmogleins, leading to skin and mucosal blisters and erosions. Scalp involvement is common but poorly characterized.
Objectives: This study investigated the prevalence, clinical patterns, immunopathological features, and correlations with disease severity and serological markers of scalp involvement.
Methods: A retrospective cohort study was conducted at the Dermatology Department, Fondazione IRCCS Policlinico San Matteo, Pavia, including patients diagnosed with pemphigus from 2012 to 2024. Scalp involvement was assessed through clinical examination and pull test. Data collected included demographics, disease subtype, anti-desmoglein antibody titers, PDAI scores, treatments, and outcomes.
Results: Among 115 patients, 57.4% had scalp involvement. Male sex was significantly associated with scalp lesions (P=0.0148). Patients with scalp involvement had higher anti-Dsg1 titers (mean 206.4 vs. 74.2 IU/mL, P<0.001) and higher PDAI scores (mean 43.9 vs. 32.1, P=0.006). Disease severity stratification also differed significantly between groups (P=0.0147). Three clinical phenotypes were identified: scaling without alopecia, crusting and erosions (further stratified into circumscribed and confluent forms), and extensive patchy alopecia, each associated with specific clinical and serological characteristics.
Conclusions: Scalp involvement is common in pemphigus and correlates with higher anti-Dsg1 titers and disease severity. Recognizing distinct scalp phenotypes may support earlier diagnosis and more targeted management.
References
Mihai S, Sitaru C. Immunopathology and molecular diagnosis of autoimmune bullous diseases. J Cell Mol Med. 2007 May-Jun;11(3):462-81. DOI: 10.1111/j.1582-4934.2007.00033.x
Sitaru C, Zillikens D. Mechanisms of blister induction by autoantibodies. Exp Dermatol. 2005 Dec;14(12):861-75. DOI: 10.1111/j.1600-0625.2005.00367.x
Pollmann R, Schmidt T, Eming R, Hertl M. Pemphigus: a Comprehensive Review on Pathogenesis, Clinical Presentation and Novel Therapeutic Approaches. Clin Rev Allergy Immunol. 2018 Feb;54(1):1-25. DOI: 10.1007/s12016-017-8662-z
Kridin K, Zelber-Sagi S, Bergman R. Pemphigus Vulgaris and Pemphigus Foliaceus: Differences in Epidemiology and Mortality. Acta Derm Venereol. 2017 Oct 2;97(9):1095-1099. DOI: 10.2340/00015555-2706
Kridin K. Pemphigus group: overview, epidemiology, mortality, and comorbidities. Immunol Res. 2018 Apr;66(2):255-270. DOI: 10.1007/s12026-018-8986-7
Ding X, Aoki V, Mascaro JM Jr, Lopez-Swiderski A, Diaz LA, Fairley JA. Mucosal and mucocutaneous (generalized) pemphigus vulgaris show distinct autoantibody profiles. J Invest Dermatol. 1997 Oct;109(4):592-6. DOI: 10.1111/1523-1747.ep12337524
Arya SR, Valand AG, Krishna K. A clinico-pathological study of 70 cases of pemphigus. Indian J Dermatol Venereol Leprol. 1999 Jul-Aug;65(4):168-71. PMID: 20921646.
Esmaili N, Chams-Davatchi C, Valikhani M, Daneshpazhooh M, Balighi K, Hallaji Z, et al. Pemphigus vulgaris in Iran: a clinical study of 140 cases. Int J Dermatol. 2007 Nov;46(11):1166-70. DOI: 10.1111/j.1365-4632.2007.03334.x
Salmanpour R, Shahkar H, Namazi MR, Rahman-Shenas MR. Epidemiology of pemphigus in south-western Iran: a 10-year retrospective study (1991-2000). Int J Dermatol. 2006 Feb;45(2):103-5. DOI: 10.1111/j.1365-4632.2004.02374.x
Daneshpazhooh M, Mahmoudi HR, Rezakhani S, Valikhani M, Naraghi ZS, Mohammadi Y, et al. Loss of normal anagen hair in pemphigus vulgaris. Clin Exp Dermatol. 2015 Jul;40(5):485-8. DOI: 10.1111/ced.12595
Xie D, Bilgic-Temel A, Abu Alrub N, Murrell DF. Alopecia in Autoimmune Blistering Diseases: A Systematic Review of Pathogenesis and Clinical Features of Disease. Skin Appendage Disord. 2019 Aug;5(5):263-275. DOI: 10.1159/000496836
Veraitch O, Ohyama M, Yamagami J, Amagai M. Alopecia as a rare but distinct manifestation of pemphigus vulgaris. J Eur Acad Dermatol Venereol. 2013 Jan;27(1):86-91. DOI: 10.1111/j.1468-3083.2011.04363.x
Eachus E, DeLamielleure LE, Mitha S, Rasul TF, Faiz A. Scalp, Oral, and Nail Pemphigus Vulgaris: Clinical Characteristics and a Review of the Literature. Cureus. 2023 Apr 30;15(4):e38334. DOI: 10.7759/cureus.38334
Sar-Pomian M, Rudnicka L, Olszewska M. Trichoscopy - a useful tool in the preliminary differential diagnosis of autoimmune bullous diseases. Int J Dermatol. 2017 Oct;56(10):996-1002. DOI: 10.1111/ijd.13725
Mergler R, Kerstan A, Schmidt E, Goebeler M, Benoit S. Atypical Clinical and Serological Manifestation of Pemphigus Vegetans: A Case Report and Review of the Literature. Case Rep Dermatol. 2017 Apr 27;9(1):121-130. DOI: 10.1159/000468919
Delmonte S, Semino MT, Parodi A, Rebora A. Normal anagen effluvium: a sign of pemphigus vulgaris. Br J Dermatol. 2000 Jun;142(6):1244-5. DOI: 10.1046/j.1365-2133.2000.03563.x
Sar-Pomian M, Czuwara J, Rudnicka L, Olszewska M. Miniaturization of sebaceous glands: A novel histopathological finding in pemphigus vulgaris and pemphigus foliaceus of the scalp. J Cutan Pathol. 2017 Oct;44(10):835-842. DOI: 10.1111/cup.12994
Maragno L, Bussato WM, Maruta CW, et al; Cooperative Group on Fogo Selvagem research. Characterization of the humoral and in situ autoantibody profile of scalp involvement in pemphigus. J Eur Acad Dermatol Venereol. 2016 Oct;30(10):e57-e59. DOI: 10.1111/jdv.13299
Sar-Pomian M, Czuwara J, Grygorowicz T, et al. Efficacy of perilesional and intralesional triamcinolone acetonide injections in pemphigus vulgaris lesions of the scalp: an effective therapeutic option. Clin Exp Dermatol. 2018 Mar;43(2):168-170. DOI: 10.1111/ced.13288
Chams-Davatchi C, Valikhani M, Daneshpazhooh M, et al. Pemphigus: analysis of 1209 cases. Int J Dermatol. 2005 Jun;44(6):470-6. DOI: 10.1111/j.1365-4632.2004.02501.x
Sar-Pomian M, Rudnicka L, Olszewska M. The Significance of Scalp Involvement in Pemphigus: A Literature Review. Biomed Res Int. 2018 Mar 25;2018:6154397. DOI: 10.1155/2018/6154397
Fard GD, Khosravi H, Ghayoumi A, et al. Anagen hair loss, anti-desmoglein 1, and pemphigus disease area index: a significant relationship? J Dtsch Dermatol Ges. 2017 Sep;15(9):946-948. DOI: 10.1111/ddg.13306
Wu H, Stanley JR, Cotsarelis G. Desmoglein isotype expression in the hair follicle and its cysts correlates with type of keratinization and degree of differentiation. J Invest Dermatol. 2003 Jun;120(6):1052-7. DOI: 10.1046/j.1523-1747.2003.12234.x. Erratum in: J Invest Dermatol. 2003 Aug;121(2):434
Hanakawa Y, Li H, Lin C, Stanley JR, Cotsarelis G. Desmogleins 1 and 3 in the companion layer anchor mouse anagen hair to the follicle. J Invest Dermatol. 2004 Nov;123(5):817-22. DOI: 10.1111/j.0022-202X.2004.23479.x
Bazzi H, Getz A, Mahoney MG, et al. Desmoglein 4 is expressed in highly differentiated keratinocytes and trichocytes in human epidermis and hair follicle. Differentiation. 2006 Mar;74(2-3):129-40. DOI: 10.1111/j.1432-0436.2006.00061.x
Kljuic A, Bazzi H, Sundberg JP, et al. Desmoglein 4 in hair follicle differentiation and epidermal adhesion: evidence from inherited hypotrichosis and acquired pemphigus vulgaris. Cell. 2003 Apr 18;113(2):249-60. DOI: 10.1016/s0092-8674(03)00273-3
Sar-Pomian M, Konop M, Gala K, Rudnicka L, Olszewska M. Scalp involvement in pemphigus: a prognostic marker. Postepy Dermatol Alergol. 2018 Jun;35(3):293-298. DOI: 10.5114/pdia.2017.71267
Starace M, Loi C, Cedirian S, et al. Trichoscopy as a monitoring tool in assessing treatment response in scalp pemphigus. J Eur Acad Dermatol Venereol. 2024 Sep;38(9):e755-e757. DOI: 10.1111/jdv.19869.
Downloads
Published
Issue
Section
License
Copyright (c) 2026 Andrea Michelerio, Stefania Barruscotti, Mara De Amici, Giacomo Fiandrino, Valeria Brazzelli, Carlo Tomasini, Camilla Vassallo
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Dermatology Practical & Conceptual applies a Creative Commons Attribution License (CCAL) to all works we publish (http://creativecommons.org/licenses/by-nc/4.0/). Authors retain the copyright for their published work.
How to Cite
Share
