A Case of Tofacitinib-Induced Lymphomatoid Papulosis With Ocular Involvement

Abstract

Janus kinase (JAK) inhibitors are being prescribed with increasing regularity in dermatology. We report on a patient who initiated treatment with tofacitinib for refractory erythema elevatum diutinum and subsequently developed a novel cutaneous outbreak characterized by firm violaceous papules on the trunk and extremities along with conjunctival injection and periorbital inflammation. Biopsy of affected tissue from both the cutaneous and ophthalmologic sources demonstrated increased numbers of CD30 + large atypical cells amid a mixed inflammatory cell infiltrate, consistent with lymphomatoid papulosis. A review of the literature reveals a plausible mechanism for the induction of persistent JAK signaling in the presence of a JAK inhibitor. We discuss this mechanism in depth because it pertains to this patient and recommend continued vigilance with the use of these immunologic agents.

JAK Inhibitors in Cutaneous T-Cell Lymphoma: Friend or Foe? A Systematic Review of the Published Literature

“…Knapp et al [ 50 ] presented a 42-year-old man with previous inflammatory arthritis and erythema elevatum diutinum (EED). After showing no improvement following the administration of several drugs such as sulfasalazine, mycophenolate mofetil, adalimumab, and dapsone, he received tofacitinib (a JAK1, 2, and 3 inhibitor).…”

“…However, besides studies describing positive effects after the use of JAK inhibitors in CTCL patients, there are also several reports on the de novo development of CTCL [ 49 , 50 , 51 , 52 ]. To our knowledge, a total of five cases have been reported, of which, three cases took multiple medications to treat arthritis [ 49 , 50 , 51 ], and two cases received GVHD treatment following allo-SCT [ 52 ].…”

“…However, besides studies describing positive effects after the use of JAK inhibitors in CTCL patients, there are also several reports on the de novo development of CTCL [ 49 , 50 , 51 , 52 ]. To our knowledge, a total of five cases have been reported, of which, three cases took multiple medications to treat arthritis [ 49 , 50 , 51 ], and two cases received GVHD treatment following allo-SCT [ 52 ]. Since all patients were receiving immunosuppressive medications, it seems that immunosuppression for a long time before taking JAK inhibitors may play a role in increasing the risk of CTCL incidents or relapse.…”