Lupus pernio responding to tofacitinib: A case report and clinical insights

Lupus pernio (LP) is a rare manifestation of chronic sarcoidosis. This presents a case of a 46-year-old female with a one-year history of a painful upper lip nodule and occasional oral ulcers. High-resolution computed tomography of the chest and abdomen was performed, revealing no clinical or radiological evidence of systemic disease. However, histopathology confirmed a diagnosis of localized sarcoidosis. Treatment with hydroxychloroquine (200 mg daily) and a tapering steroid was initially prescribed. However, the patient experienced a flare of skin lesions when steroids were tapered and stopped at six weeks. Methotrexate (10 mg per week) was introduced instead of hydroxychloroquine along with a tapering steroid regimen. Despite this, another flare occurred upon steroid tapering and discontinuation. Consequently, tofacitinib (5 mg daily) was initiated along with steroids, which were gradually tapered. The patient showed significant improvement, with resolution of both the skin lesion and oral ulcers. Steroids were discontinued at sixth week, and more than a month after their cessation, no further flares were observed. This case underscores the importance of early recognition and highlights the efficacy of a tailored immunosuppressive approach, including tofacitinib, in managing steroid-dependent LP.