Clinical Images: Chronic, refractory childhood‐onset cutaneous lupus erythematosus of the face responsive to lenalidomide

Abstract

Clinical Images: Chronic, refractory childhood-onset cutaneous lupus erythematosus of the face responsive to lenalidomideThe patient, an 11-year-old girl with anti-Ro/SSA and hypergammaglobulinemia, presented in 2013 with prominent facial eruption consisting of red to purple nodules concentrated on the forehead, around the eyebrows, and on the cheeks that resulted in significant hyperpigmentation, scarring, and atrophy. This pattern was consistent with a diagnosis of cutaneous lupus (A); the patient had no signs of systemic lupus erythematosus. The rash was refractory to hydroxychloroquine (HCQ), topical calcineurin inhibitor, topical steroids, intralesional steroids, oral steroids, dapsone, azathioprine, and mycophenolate. The patient's course was complicated by intermittent follow-up and frequent periods of not receiving medications. Systemic therapies were discontinued by the family prior to the COVID-19 pandemic. Biopsy was repeated in September 2020 because of ongoing disease activity and diagnostic uncertainty. Histologic examination revealed a superficial and deep perivascular and periadnexal lymphohistiocytic infiltrate associated with mild overlying interface changes, telangiectasias, and increased dermal mucin consistent with chronic cutaneous lupus (B). Lenalidomide (LND) has been found to be effective and generally well tolerated in refractory cases of cutaneous lupus in adults and adolescents (1-3). The typical starting dosage is 5 mg per day for both age groups. In February 2021, LND 2.5 mg was started, with a plan to titrate up based on clinical response, in combination with HCQ. At most recent follow-up in May 2021, the patient had significant improvement in cutaneous lesions and has remained on the initial dose (C). The patient has tolerated LND thus far without apparent adverse effects, such as cytopenias or neuropathy. Due to risk of teratogenicity, monthly urine pregnancy tests have been required since the patient reached menarche.