Rilzabrutinib-induced transition from pemphigus vulgaris to pemphigus foliaceous: case report and review of the literature
Accepted: 3 October 2024
Supplementary Materials: 33
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
The discovery of the role of Bruton’s Tyrosine Kinase (BTK) in inflammation and autoimmunity has recently led to the development of BTK inhibitors for the treatment of autoimmune diseases, including pemphigus vulgaris. We herein present the case of a patient affected by pemphigus vulgaris, refractory to conventional immunosuppressive therapies and to multiple courses of rituximab, who was treated with rilzabrutinib and achieved disease control, but whose immunological profile switched from pemphigus vulgaris to pemphigus foliaceus after drug discontinuation. Furthermore, we review the literature in order to better characterize the phenotypic transitions from pemphigus vulgaris to pemphigus foliaceus reported so far. The factors underlying this transition are largely unknown, although it has been postulated that immunosuppressive therapies may be more effective against anti-desmoglein 3 antibodies compared to anti-desmoglein 1. However, further studies are needed to better define the effect of rilzabrutinib (and immunosuppressive therapies in general) on anti-desmoglein 1 and anti-desmoglein 3 antibodies.
Patsatsi A, Murrell DF. Bruton Tyrosine Kinase Inhibition and Its Role as an Emerging Treatment in Pemphigus. Front Med (Lausanne) 2021;8:708071. DOI: https://doi.org/10.3389/fmed.2021.708071
Lee A, Sandhu S, Imlay-Gillespie L, et al. Successful use of Bruton’s kinase inhibitor, ibrutinib, to control paraneoplastic pemphigus in a patient with paraneoplastic autoimmune multiorgan syndrome and chronic lymphocytic leukaemia. Australas J Dermatol 2017;58:e240-2. DOI: https://doi.org/10.1111/ajd.12615
Murrell DF, Patsatsi A, Stavropoulos P, et al. Proof of concept for the clinical effects of oral rilzabrutinib, the first Bruton tyrosine kinase inhibitor for pemphigus vulgaris: the phase II BELIEVE study*. Br J Dermatol 2021;185:745-55. DOI: https://doi.org/10.1111/bjd.20431
Amagai M. Bolognia JL, Schaffer JV, Cerroni L. Dermatology. 4th ed. New York: Elsevier; 2018. Pemphigus; pp. 494–509.
Amagai M, Tsunoda K, Zillikens D, et al. The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile. J Am Acad Dermatol 1999;40:167-70. DOI: https://doi.org/10.1016/S0190-9622(99)70183-0
Komai A, Amagai M, Ishii K, et al. The clinical transition between pemphigus foliaceus and pemphigus vulgaris correlates well with the changes in autoantibody profile assessed by an enzyme-linked immunosorbent assay. Br J Dermatol 2001;144:1177-82. DOI: https://doi.org/10.1046/j.1365-2133.2001.04227.x
Ishii K, Amagai M, Ohata Y, et al. Development of pemphigus vulgaris in a patient with pemphigus foliaceus: Antidesmoglein antibody profile shift confirmed by enzyme-linked immunosorbent assay. J Am Acad Dermatol 2000;42:859-61. DOI: https://doi.org/10.1016/S0190-9622(00)90253-6
Awazawa R, Yamamoto Y, Gushi M, et al. Case of pemphigus foliaceus that shifted into pemphigus vulgaris after adrenal tumor resection. J Dermatol 2007;34:549-55. DOI: https://doi.org/10.1111/j.1346-8138.2007.00329.x
Wang HH, Liu CW, Li YC, et al. Efficacy of rituximab for pemphigus: a systematic review and meta-analysis of different regimens. Acta Derm Venereol 2015;95:928-32. DOI: https://doi.org/10.2340/00015555-2116
Russo I, Miotto S, Saponeri A, et al. Ultra-low dose rituximab for refractory pemghigus vulgaris: a pilot study. Expert Opin Biol Ther 2020;20:673-8. DOI: https://doi.org/10.1080/14712598.2020.1727440
Iwatsuki K, Takigawa M, Hashimoto T, et al. Can pemphigus vulgaris become pemphigus foliaceus? J Am Acad Dermatol 1991;25:797-800. DOI: https://doi.org/10.1016/S0190-9622(08)80971-1
Kawana S. Changes in Clinical Features, Histologic Findings, and Antigen Profiles With Development of Pemphigus Foliaceus From Pemphigus Vulgaris. Arch Dermatol 1994;130:1534. DOI: https://doi.org/10.1001/archderm.1994.01690120070010
Chang SN, Kim SC, Lee IJ, et al. Transition from pemphigus vulgaris to pemphigus foliaceus. Br J Dermatol. 1997;137:303-5.
Kimoto M, Ohyama M, Hata Y, et al. A Case of Pemphigus foliaceus Which Occurred after Five Years of Remission from Pemphigus vulgaris. Dermatology 2001;203:174-6. DOI: https://doi.org/10.1159/000051737
Harman KE, Gratian MJ, Shirlaw PJ, et al. The transition of pemphigus vulgaris into pemphigus foliaceus: a reflection of changing desmoglein 1 and 3 autoantibody levels in pemphigus vulgaris. Br J Dermatol 2002;146:684-7. DOI: https://doi.org/10.1046/j.1365-2133.2002.04608.x
Toth GG, Pas HH, Jonkman MF. Transition of pemphigus vulgaris into pemphigus foliaceus confirmed by antidesmoglein ELISA profile. Int J Dermatol 2002;41:525-7. DOI: https://doi.org/10.1046/j.1365-4362.2002.15452.x
Tsuji Y, Kawashima T, Yokota K, et al. Clinical and Serological Transition From Pemphigus Vulgaris to Pemphigus Foliaceus Demonstrated by Desmoglein ELISA System. Arch Dermatol 2002;138. DOI: https://doi.org/10.1001/archderm.138.1.95
Ng PPL, Thng STG. Three Cases of Transition from Pemphigus vulgaris to Pemphigus foliaceus Confirmed by Desmoglein ELISA. Dermatology 2005;210:319-21. DOI: https://doi.org/10.1159/000084757
Lévy-Sitbon C, Reguiaï Z, Durlach A, et al. Transition phénotypique d’un pemphigus vulgaire en pemphigus superficiel. Ann Dermatol Vénéréologie 2013;140:788-92. DOI: https://doi.org/10.1016/j.annder.2013.07.013
España A, Koga H, Suárez-Fernández R, et al. Antibodies to the amino-terminal domain of desmoglein 1 are retained during transition from pemphigus vulgaris to pemphigus foliaceus. Eur J Dermatol 2014;24:174-9. DOI: https://doi.org/10.1684/ejd.2014.2277
Ito T, Moriuchi R, Kikuchi K, et al. Rapid transition from pemphigus vulgaris to pemphigus foliaceus. J Eur Acad Dermatol Venereol 2016;30:455-7. DOI: https://doi.org/10.1111/jdv.12832
Mohammadrezaee M, Etesami I, Mahmoudi H, et al. Transition between pemphigus vulgaris and pemphigus foliaceus: a 10‐year follow‐up study. JDDG J Dtsch Dermatol Ges 2020;18:1302-4. DOI: https://doi.org/10.1111/ddg.14226
Pigozzi B, Peserico A, Schiesari L, Alaibac M. Pemphigus foliaceus evolving into pemphigus vulgaris: a probable example of ?intermolecular epitope spreading? confirmed by enzyme-linked immunosorbent assay study. J Eur Acad Dermatol Venereol 2008;22:242-4. DOI: https://doi.org/10.1111/j.1468-3083.2007.02298.x
Copyright (c) 2024 the Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
PAGEPress has chosen to apply the Creative Commons Attribution NonCommercial 4.0 International License (CC BY-NC 4.0) to all manuscripts to be published.