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CC BY-NC-ND 4.0 · Z Gastroenterol 2024; 62(09): 1384-1388
DOI: 10.1055/a-2300-0375
Kasuistik

Remission of refractory esophageal lichen planus induced by tofacitinib

Remission eines refraktären Lichen planus im Ösophagus durch Tofacitinib
Valentina Bieneck
1   Department of Dermatology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany (Ringgold ID: RIN88751)
,
Annegrit Decker 
2   Department of Medicine II, Gastroenterology, Hepatology, Endocrinology and Infectious Diseases, Medical Center, University of Freiburg, Freiburg, Germany (Ringgold ID: RIN88751)
,
Annette Schmitt-Graeff 
3   University of Freiburg Faculty of Medicine, Freiburg, Germany (Ringgold ID: RIN88751)
,
Wolfgang Kreisel 
2   Department of Medicine II, Gastroenterology, Hepatology, Endocrinology and Infectious Diseases, Medical Center, University of Freiburg, Freiburg, Germany (Ringgold ID: RIN88751)
,
Franziska Schauer 
1   Department of Dermatology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany (Ringgold ID: RIN88751)
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Abstract

As of now, there exists no established therapy for ELP. Retinoids, which are standard in treating cutaneous LP, do not exhibit positive effects in ELP. While topical glucocorticosteroids often yield favorable responses in esophageal inflammation, some cases prove recalcitrant or refractory. In such instances, various immunosuppressive therapies have been attempted with variable success.

This report details a severe case of ELP that showed resistance to prednisolone, acitretin, alitretinoin, adalimumab, tacrolimus, hydroxychloroquine plus mycophenolate mofetil, and cyclophosphamide. The initiation of the JAK inhibitor tofacitinib induced an impressive clinical, endoscopic, and histological remission. This positive response to a JAK inhibitor is discussed in the context of our evolving understanding of the immune-mediated pathogenesis of this disease.

Zusammenfassung

Eine Beteiligung des Ösophagus (ELP) ist eine selten diagnostizierte Manifestation eines Lichen planus, einer gut bekannten, wahrscheinlich autoimmun-vermittelten Hauterkrankung. In den letzten Jahren wurden Kriterien zur Diagnose vorgestellt: Bei der Endoskopie sieht man eine Schleimhautablösung, ein Einreißen der Schleimhaut, eine Trachealisierung ähnlich wie bei der eosinophilen Ösophagitis. Die Histologie zeigt eine bandförmige Infiltration mit T-Lymphozyten im Bereich der Basalmembran mit Übergreifen in das Epithelium und einer Ablösung der Schleimhaut, Apoptosen der Epithelzellen, und eine Hyperkeratose, auf deren Boden sich ein Plattenepithel-Karzinom entwickeln kann. Führendes klinisches Symptom ist eine Dysphagie. Symptomfreie Verläufe kommen vor, aber auch Fälle mit oberer gastrointestinaler Blutung. Eine etablierte Therapie des ELP gibt es noch nicht. Meist spricht die Erkrankung auf topische Kortikosteroide an. In schweren Fällen wurden verschiedene immunsuppressive Therapien angewandt.

Wir beschreiben eine Patientin mit einem schweren ELP, die auf eine Therapie mit topischen oder systemischen Kortikosteroiden, Acitretin, Alitretinoin, Adalimumab, Tacrolimus, Hydroxychloroquin kombiniert mit Mycophenolatmofetil und Cylophosphamid nicht oder kaum ansprach. Nach Gabe des JAK-Inhibitors Tofacitinib kam es zu einer eindrucksvollen klinischen, endoskopischen und histologischen Remission. Wir diskutieren diesen positiven Effekt eines JAK-Inhibitors bei einem ELP in Zusammenhang mit neuen Erkenntnissen über die Immunpathogenese des Lichen planus.

Keywords

lichen planus - JAK-inhibitor - esophagus - autoimmunity - dermatology - mucosal detachment - endoscopy

Schlüsselwörter

Lichen ruber - JAK Inhibitor - Ösophagus - Autoimmunität - Dermatologie - Mucosale Spaltbildung - Endoskopie


Publication History

Received: 18 December 2023

Article published online:
25 June 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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  • References

  • 1 González-Moles MÁ, Warnakulasuriya S, González-Ruiz I. et al. Worldwide prevalence of oral lichen planus: A systematic review and meta-analysis. Oral Dis 2021; 27: 813-828
    CrossrefPubMedSearch in Google Scholar
  • 2 Li C, Tang X, Zheng X. et al. Global Prevalence and Incidence Estimates of Oral Lichen Planus: A Systematic Review and Meta-analysis. JAMA Dermatol 2020; 156: 172-181
    CrossrefPubMedSearch in Google Scholar
  • 3 Le Cleach L, Chosidow O. Clinical practice. Lichen planus. N Engl J Med 2012; 366: 723-732
    CrossrefPubMedSearch in Google Scholar
  • 4 Harewood GC, Murray JA, Cameron AJ. Esophageal lichen planus: the Mayo Clinic experience. Dis Esophagus 1999; 12: 309-311
    CrossrefPubMedSearch in Google Scholar
  • 5 Quispel R, van Boxel OS, Schipper ME. et al. High prevalence of esophageal involvement in lichen planus: a study using magnification chromoendoscopy. Endoscopy 2009; 41: 187-193
    Thieme ConnectPubMedSearch in Google Scholar
  • 6 Katzka DA, Smyrk TC, Bruce AJ. et al. Variations in presentations of esophageal involvement in lichen planus. Clin Gastroenterol Hepatol 2010; 8: 777-782
    CrossrefPubMedSearch in Google Scholar
  • 7 Kern JS, Technau-Hafsi K, Schwacha H. et al. Esophageal involvement is frequent in lichen planus: study in 32 patients with suggestion of clinicopathologic diagnostic criteria and therapeutic implications. European Journal of Gastroenterology & Hepatology 2016; 28: 1374-1382
    CrossrefPubMedSearch in Google Scholar
  • 8 Schauer F, Monasterio C, Technau-Hafsi K. et al. Esophageal lichen planus: towards diagnosis of an underdiagnosed disease. Scandinavian Journal of Gastroenterology 2019; 54: 1189-1198
    CrossrefPubMedSearch in Google Scholar
  • 9 Monasterio C, Decker A, Schauer F. et al. Der Lichen planus des Ösophagus – Eine unterschätzte Erkrankung. Z Gastroenterol 2021; 59: 460-469
    Thieme ConnectPubMedSearch in Google Scholar
  • 10 Decker A, Schauer F, Lazaro A. et al. Esophageal lichen planus: Current knowledge, challenges and future perspectives. World J Gastroenterol 2022; 28: 5893-5909
    CrossrefPubMedSearch in Google Scholar
  • 11 Jacobs JW, Kukreja K, Camisa C. et al. Demystifying Esophageal Lichen Planus: A Comprehensive Review of a Rare Disease You Will See in Practice. Am J Gastroenterol 2022; 117: 70-77
    CrossrefPubMedSearch in Google Scholar
  • 12 Sengupta N, Tapper EB, Corban C. et al. The clinical predictors of aetiology and complications among 173 patients presenting to the Emergency Department with oesophageal food bolus impaction from 2004–2014. Aliment Pharmacol Ther 2015; 42: 91-98
    CrossrefPubMedSearch in Google Scholar
  • 13 Bombeccari GP, Pallotti F, Guzzi G. et al. Oral-esophageal lichen planus associated with oral squamous cell carcinoma. Indian J Dermatol Venereol Leprol 2008; 74: 509-511
    CrossrefPubMedSearch in Google Scholar
  • 14 Ravi K, Codipilly DC, Sunjaya D. et al. Esophageal Lichen Planus Is Associated With a Significant Increase in Risk of Squamous Cell Carcinoma. Clin Gastroenterol Hepatol 2019; 17: 1902-1903.e1
    CrossrefPubMedSearch in Google Scholar
  • 15 Ioannides D, Vakirlis E, Kemeny L. et al. European S1 guidelines on the management of lichen planus: a cooperation of the European Dermatology Forum with the European Academy of Dermatology and Venereology. J Eur Acad Dermatol Venereol 2020; 34: 1403-1414
    CrossrefPubMedSearch in Google Scholar
  • 16 Solimani F, Forchhammer S, Schloegl A. et al. Lichen planus – a clinical guide. JDDG: Journal der Deutschen Dermatologischen Gesellschaft 2021; 19: 864-882
    CrossrefPubMedSearch in Google Scholar
  • 17 Eyerich K, Eyerich S. Immune response patterns in non-communicable inflammatory skin diseases. J Eur Acad Dermatol Venereol 2018; 32: 692-703
    CrossrefPubMedSearch in Google Scholar
  • 18 Barrat FJ, Crow MK, Ivashkiv LB. Interferon target-gene expression and epigenomic signatures in health and disease. Nat Immunol 2019; 20: 1574-1583
    CrossrefPubMedSearch in Google Scholar
  • 19 Damsky W, King BA. JAK inhibitors in dermatology: the promise of a new drug class. J Am Acad Dermatol 2017; 76: 736-744
    CrossrefPubMedSearch in Google Scholar
  • 20 Damsky W, Wang A, Olamiju B. et al. Treatment of severe lichen planus with the JAK inhibitor tofacitinib. J Allergy Clin Immunol 2020; 145: 1708-1710.e2
    CrossrefPubMedSearch in Google Scholar
  • 21 Shao S, Tsoi LC, Sarkar MK. et al. IFN-γ enhances cell-mediated cytotoxicity against keratinocytes via JAK2/STAT1 in lichen planus. Sci Transl Med 2019; 11: eaav7561
    CrossrefPubMedSearch in Google Scholar
  • 22 Brumfiel CM, Patel MH, Severson KJ. et al. Ruxolitinib Cream in the Treatment of Cutaneous Lichen Planus: A Prospective, Open-Label Study. J Invest Dermatol 2022; 142: 2109-2116.e4
    CrossrefPubMedSearch in Google Scholar
  • 23 Solimani F, Meier K, Ghoreschi K. Emerging Topical and Systemic JAK Inhibitors in Dermatology. Front Immunol 2019; 10: 2847
    CrossrefPubMedSearch in Google Scholar
  • 24 Didona D, Caposiena Caro RD, Sequeira Santos AM. et al. Therapeutic strategies for oral lichen planus: State of the art and new insights. Front Med (Lausanne) 2022; 9: 997190
    CrossrefPubMedSearch in Google Scholar
  • 25 Vičić M, Hlača N, Kaštelan M. et al. Comprehensive Insight into Lichen Planus Immunopathogenesis. Int J Mol Sci 2023; 24: 3038
    CrossrefPubMedSearch in Google Scholar
  • 26 Abduelmula A, Bagit A, Mufti A. et al. The Use of Janus Kinase Inhibitors for Lichen Planus: An Evidence-Based Review. J Cutan Med Surg 2023; 27: 271-276
    CrossrefPubMedSearch in Google Scholar
  • 27 Aihie O, Dyer JA. JAK Inhibitors: A New Weapon in the Skin Care Providers’ Arsenal. Mo Med 2023; 120: 45-48
    PubMedSearch in Google Scholar
  • 28 Huang D, Zhang Y, Kong L. et al. Janus kinase inhibitors in autoimmune bullous diseases. Front Immunol 2023; 14: 1220887
    CrossrefPubMedSearch in Google Scholar
  • 29 Mpakogiannis K, Fousekis FS, Christodoulou DK. et al. The current role of Tofacitinib in acute severe ulcerative colitis in adult patients: A systematic review. Dig Liver Dis 2023; 55: 1311-1317
    CrossrefPubMedSearch in Google Scholar
  • 30 Macaluso FS, D’Antonio E, Fries W. et al. Safety and effectiveness of tofacitinib in ulcerative colitis: Data from TOFA-UC, a SN-IBD study. Dig Liver Dis 2023;
    CrossrefPubMedSearch in Google Scholar
  • 31 Tursi A, Mocci G, Cingolani L. et al. Use of tofacitinib as first or second-line therapy is associated with better outcomes in patients with ulcerative colitis: data from a real-world study. Expert Opin Pharmacother 2023; 24: 1649-1656
    CrossrefPubMedSearch in Google Scholar
  • 32 Irving PM, Leung Y, Dubinsky MC. Review article: guide to tofacitinib dosing in patients with ulcerative colitis. Aliment Pharmacol Ther 2022; 56: 1131-1145
    CrossrefPubMedSearch in Google Scholar
  • 33 Kooybaran NR, Petzold G, Schön MP. et al. Esophageal lichen planus successfully treated with the JAK1/3 inhibitor tofacitinib. J Dtsch Dermatol Ges 2022; 20: 858-860
    CrossrefPubMedSearch in Google Scholar
  • 34 Kozlov M, Levit EK, Silvers DN. et al. Severe Esophageal Lichen Planus Treated With Tofacitinib. Cutis 2023; 111: 155-163
    CrossrefPubMedSearch in Google Scholar