Remission of refractory esophageal lichen planus induced by tofacitinib

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

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