Inflammatory Arthritis, Sacroiliitis, and Morphea: Evidence of a Systemic Inflammatory Disease

Morphea is a skin disease characterized by local skin inflammation and fibrosis. Extracutaneous manifestations have been described with this disease including inflammatory arthritis. We describe a case of morphea who developed inflammatory polyarthritis and sacroiliitis coincident with new skin lesions. 1. Introduction Morphea or localized scleroderma is an idiopathic disorder characterized by local inflammation followed by fibrotic changes in the skin and subcutaneous tissues. Joint involvement has traditionally been thought to be related to the extent of skin disease, manifesting as arthralgia, impaired joint mobility, or joint contracture [1, 2]. We describe a patient who developed inflammatory polyarthritis and sacroiliitis with worsening of her morphea. 2. Case A 49-year-old female presented to the Toronto Scleroderma Program with a history of biopsy-proven circumscribed morphea over the abdomen diagnosed in 1994. At that time, her lesions improved spontaneously with no need for treatment. In 1998, after suffering a motor vehicle accident necessitating back surgery, she developed another circumscribed lesion around the surgical scar, which was managed conservatively. In 2008, the patient started to notice the development of multiple progressive linear lesions involving the upper and lower limbs with skin hardening and hyperpigmentation, but sparing the hands and feet. In June 2011, she noted progressive spreading and thickening of these lesions. Over the same time period, she developed swelling and pain involving the metacarpophalangeal (MCP), proximal interphalangeal (PIP) joints and left knee. Additionally, she developed inflammatory back pain with more than 1 hour of morning stiffness. The patient had recurrent attacks of uveitis, treated with local steroids, with no visual sequelae. The patient did not exhibit any symptoms suggestive of systemic sclerosis, systemic lupus erythematosus (SLE), or inflammatory bowel disease. Her family history was significant for psoriasis in her father. In October 2011, methotrexate 15？mg/week was initiated with subsequent improvement of her peripheral joint pain and swelling, but there was no significant response in the axial pain and stiffness. She was first seen in our clinic in June 2012. Her examination revealed linear morphea involving all 4 limbs with active inflammation and chronic atrophic changes in the abdominal and back areas but no psoriatic lesions. She had tenderness and swelling involving the MCP, PIP, and left knee joints with swollen and tender joint counts of 5 and 6, respectively. Axial