LAUSR

Dear Editor, A 62-year-old woman presented to the rheumatology outpatients clinic with a 2-month history of symmetrical inflammatory polyarthritis affecting the metacarpophalangeal joints (MCPJs) and proximal interphalangeal joints (PIPJs). Other complaints included generalized fatigue and mild myalgia without weakness. There were no features of a connective tissue disorder. The patient had been started on prednisone by her family doctor with minimal relief of symptoms. The patient was known to be a carrier of the BRCA1 tumor suppressor gene and had undergone prophylactic oophorectomy 15 years prior. In 2013 a routine mammogram identified an early localized breast lump which was treated with a bilateral mastectomy. Histology confirmed estrogen and progesterone receptor (ER/PR) and (HER) Human epidermal growth factor receptor negativity so there was no role for tamoxifen or an aromatase inhibitor. Chemotherapy was felt to be of minimal benefit so was not administered. Past medical history was otherwise unremarkable. Examination revealed tenderness across multiple MCPJs and PIPJs with mild synovitis of a number of joints. An unusual feature was non-tender, purple erythematous nodularity of the palms without flexor tenosynovitis (Fig. 1). There was no sclerodactyl, nail bed changes or telangiectasia. General examination was unremarkable. Investigations included unremarkable inflammatory markers and negative rheumatoid factor (RF), anticyclic citrullinated peptide antibody (anti-CCP), antinuclear antibodies (ANA), extractable nuclear antigen (ENA) and double-stranded DNA. X-rays of the hands showed changes of early nodal osteoarthritis. The initial diagnosis was of a seronegative arthritis with atypical features. Methotrexate was started and prednisone was weaned. Over the following 6 weeks the patient developed rapidly progressive palmar fasciitis with marked fixed flexion deformities. She was unable to make a fist and had difficulty performing her job as a receptionist. An area of erythema developed on the reconstructed breast so a tissue biopsy was performed. This revealed recurrence of initial ER/ PR/HER negative breast cancer within the dermal lymphatics. A diagnosis of paraneoplastic palmer fasciitis and polyarthritis syndrome was made. Methotrexate was stopped and ongoing symptoms were managed with anti-inflammatories. The patient was referred to the oncology service for further management. A re-staging computed tomography (CT) scan and nuclear medicine bone scan did not suggest metastatic disease. Adriamycin and cyclophosphamide chemotherapy was administered prior to further surgery. With chemotherapy the patient’s palmar fasciitis and polyarthritis progression halted, although the palmar contractures have not at this stage resolved.