Practice CMAJ

A60-year-old man born in Canada wasreferred to our clinic because of arthri-tis, a progressive maculopapular rash and severe peripheral neuropathy. He had a his-tory of hypothyroidism and hypogonadism, com-pletely corrected with levothyroxine and testos-terone, respectively. He had been evaluated five years earlier for symmetrical arthritis of the small joints in both hands that was associated with morning stiffness. At the time, his rheuma-toid factor and cyclic citrullinated peptide anti-body (anti-CCP) test results were negative. Seronegative rheumatoid arthritis had been diag-nosed and oral treatment with prednisone and methotrexate started. The patient had stopped the methotrexate because of lack of effect. A few months after the start of the immunosup-pressive agents, a progressive erythematous macu-lopapular rash had developed on the patient’s face, trunk and limbs. A skin biopsy had shown the presence of granulomas. Interstitial granulomatous dermatitis associated with rheumatoid disease had been diagnosed and topical glucocorticoid treat-ment prescribed. However, the rash continued to progress slowly over the years. About one year after the initial presentation and six months before referral to our clinic, the patient reported edema of his limbs and glove–stocking sensory loss associated with burning pain. The neurologic symptoms were disabling and kept him from working as a car mechanic. A neurologist evaluated the patient and performed electromyog-raphy, which showed a mixed peripheral neuropa-thy. All investigations to elucidate the cause of the neuropathy were negative, and the neurologist diagnosed familial sensitivomotor polyneuropathy. The patient was subsequently referred to our clinic by his family physician for further investigation. On physical examination, he had no fever. He had a confluent erythematous, maculopapular, hypoesthetic rash on his face, trunk and limbs that spared the warmer areas of the body (Figure 1). Other dermatologic findings included thickening of the skin over his nose and loss of his eyebrows. What is your call? Arthritis, progressive maculopapular rash and severe peripheral neuropathy in a 60-year-old ma