Understanding the Role of Type I Interferons in Cutaneous Lupus and Dermatomyositis: Toward Better Therapeutics.

A 29-year-old female presented to a rheumatology-dermatology clinic with a pruritic rash that began 6 months prior, after a viral illness. She had previously been diagnosed with eczema and treated with antihistamines and topical steroids without improvement. She also noted fatigue, hair loss, and severe scalp pruritus. Physical examination was notable for violaceous periorbital edema, scaly erythematous papules on the metacarpophalangeal joints of bilateral hands, dilated capillaries of the proximal nail folds, scaly plaques on bilateral elbows, and excoriated erythematous plaques on upper chest, back and hips. The patient reported no muscle weakness, and strength testing and creatinine phosphokinase were normal. Magnetic resonance imaging of the thigh showed no evidence of inflammation or edema. Antibody testing was negative. A diagnosis of clinically amyopathic dermatomyositis was made. Computed tomography scans of the chest, abdomen and pelvis, colonoscopy, and mammogram showed no evidence of cancer. The patient was initiated on methotrexate. Her cutaneous manifestations persisted with debilitating intractable pruritus, and thus, she was transitioned to mycophenolate mofetil, again with minimal improvement. Intravenous immunoglobulin was not approved by insurance given the lack of muscle involvement in her disease. This patient's case highlights a common clinical scenario in rheumatology and dermatology and raises several important issues related to the immunologic underpinnings of cutaneous lupus erythematosus (CLE) and dermatomyositis (DM): What is the role of type I interferon (IFN) in triggering skin disease in CLE and DM? What is the role of IFN in the pathogenesis of skin inflammation in CLE and DM? Can we apply what we know about IFN-targeted therapeutics in CLE and DM to develop better treatments for skin disease?
(© 2024 The Author(s). Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)