Remission of Bullous Pemphigoid in a Patient Treated with Upadacitinib: A Case Report
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Keywords
bullous pemphigoid, JAK inhibitor, upadacitinib, refractory, treatment-resistant, BP
Abstract
Bullous pemphigoid (BP) is a chronic autoimmune blistering disorder that often requires complex medical management. Cases of recalcitrant BP that do not respond to first line agents pose a therapeutic dilemma. Herein, we present a case of recalcitrant BP successfully treated with an oral janus kinase inhibitor, upadacitinib, after limited response with prednisone tapering, doxycycline, dupilumab and omalizumab. We observed substantial improvement following the initiation of upadacitinib after two months, complete remission after six months, and sustained remission one-year post-therapy. This case highlights the emerging potential of JAK inhibitors in managing refractory BP.
References
2. Borradori L, Van Beek N, Feliciani C, et al. Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2022 Oct;36(10):1689-1704. doi: 10.1111/jdv.18220.
3. Williams HC, Wojnarowska F, Kirtschig G, et al.; UK Dermatology Clinical Trials Network BLISTER Study Group. Doxycycline versus prednisolone as an initial treatment strategy for bullous pemphigoid: a pragmatic, non-inferiority, randomised controlled trial. Lancet. 2017 Apr;389(10079):1630-1638. doi: 10.1016/S0140-6736(17)30560-3.
4. Sticherling M, Franke A, Aberer E, et al. An open, multicentre, randomized clinical study in patients with bullous pemphigoid comparing methylprednisolone and azathioprine with methylprednisolone and dapsone. Br J Dermatol. 2017 Nov;177(5):1299-1305. doi: 10.1111/bjd.15649.
5. Zhao L, Wang Q, Liang G, et al. Evaluation of Dupilumab in Patients With Bullous Pemphigoid. JAMA Dermatol. 2023 Sept;159(9):953-960. doi: 10.1001/jamadermatol.2023.2428.
6. Xiao Y, Xiang H, Li W. Concurrent bullous pemphigoid and plaque psoriasis successfully treated with Janus kinase inhibitor Baricitinib. Dermatol Ther. 2022 Oct;35(10):e15754. Doi: 10.1111/dth.15754.
7. Nash D, Kirchhof MG. Bullous pemphigoid treated with Janus kinase inhibitor upadacitinib. JAAD Case Rep. 2022 Dec;32:81-83. doi: 10.1016/j.jdcr.2022.12.006.
8. Gresham LM, Kirchhof MG. A case of drug-induced bullous pemphigoid secondary to immunotherapy treated with upadacitinib: A case report. SAGE Open Med Case Rep. 2023 Mar;11:2050313X231160926. doi: 10.1177/2050313X231160926.
9. Samuel C, Cornman H, Kambala A, Kwatra SG. A Review on the Safety of Using JAK Inhibitors in Dermatology: Clinical and Laboratory Monitoring. Dermatol Ther (Heidelb). 2023;13(3):729-749. doi: 10.1007/s13555-023-00892-5.
10. Amara, S., Sapoznik, A., Guenin, S. , et al. 2024. Off-Label Uses of Upadacitinib. SKIN The Journal of Cutaneous Medicine. 8, 4 (Jul. 2024), 1614–1643. doi:https://doi.org/10.25251/skin.8.4.1.
11. Juczynska K, Wozniacka A, Waszczykowska E, et al. Expression of the JAK/STAT Signaling Pathway in Bullous Pemphigoid and Dermatitis Herpetiformis. Mediators Inflamm. 2017;2017:6716419. doi: 10.1155/2017/6716419.
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