A 56-year-old female with no history of arthritis presented with a 2-month history of a diffuse, pruritic painful papulonodular skin eruption, diffuse myalgias, and symmetric polyarthritis of the hands, shoulders, knees, and ankles.The patient was started on oral prednisone 40 mg daily for a few days and uptitrated to 60 mg daily for 2 weeks, with development of additional painful nodules on her ears and nares and persistent joint pain. The nodules were treated with intralesional triamcinolone acetonide 40 mg/ml, followed by clobetasol 0.05% ointment without improvement. Cyclosporine 5 mg/kg/day was then added in addition to 40 mg of prednisone daily while laboratory evaluation was ongoing and before diagnosis was definitive. On this regimen, she continued to develop painful nodules, resulting in the patient's self-discontinuation of cyclosporine. Joint and skin disease flared, impairing sleep and activities of daily living any time prednisone was tapered to 30 mg daily. To confirm the diagnosis, punch biopsies of the back and wrist were performed.Past medical, social, and family history She had no significant past medical history. The patient was a former smoker who quit 1 month prior to symptom onset. She did not use alcohol or illicit drugs. She worked as a nursing supervisor but had recently quit due to her debilitating arthritis. She had a family history of esophageal cancer, lung cancer, and colon cancer. There was no family history of autoimmune or rheumatologic disorders.
Review of systemsShe denied fevers, chills, unintentional weight loss, oral ulcers, Raynaud's phenomenon, photosensitivity, chest pain, shortness of breath, abdominal pain, weakness, or neurologic symptoms.
Physical examinationThe patient's vital signs were within normal limits. She was well-developed and in no acute distress. There was cobblestoning of the right posterior buccal mucosa without any associated tongue lesions. The lateral bridge of the nose and nares were firm with overlying violaceous papulonodules. Erythematous, thin stippled plaques were present over the left forehead, temporal scalp, bilateral lateral neck, and posterior neck. There were diffuse cherry-red and violaceous papulonodules on the bilateral elbows, sparing the olecranon, and extending linearly along the forearms. Similar papulonodules were present on the palms, knees, anterior thighs, periungual regions, metacarpophalangeal (MCP), proximal interphalangeal (PIP), and distal interphalangeal (DIP) joints ( Figure 1A). Inspection of the central back showed coalescent, erythematous flagellate plaques (Figure 1B). The musculoskeletal examination was notable for tenderness to palpation of the bilateral glenohumeral joints, limited bilateral shoulder forward flexion and abduction, tenderness to palpation of the bilateral finger joints with subtle swelling of the PIP joints, inability to make a fist bilaterally, and tenderness to palpation of the metatarsophalangeal joints without synovitis. There was no lymphadenopathy in the cervical, axillary, epitrochlear, and in...