The skin as the first sign of a rare complication of rheumatoid arthritis

Excerpt

A 45-year-old female was admitted for non-painful ulcers involving face, trunk, and limbs. Lesions started 3 days before as red macules evolving into ulcers with a black eschar. The patient also reported fever, rhinorrhea, and cough. She had a 13-year history of seropositive and erosive rheumatoid arthritis (RA) treated with a low dose of prednisolone (5–7.5 mg/day since diagnosis). She had an irregular follow-up and had never used any disease-modifying antirheumatic drug. On physical examination, several ulcerations were observed, surrounded by erythema and covered by a necro-hemorrhagic crust (Fig. 1). Examination of the nose showed crusting and mucous rhinorrhea. Laboratory results revealed leukopenia (white blood cell count 2200/mm³) with an absolute neutrophil count of 230/mm³. C-reactive protein was 230 mg/L. The levels of rheumatoid factor and anti-cyclic citrullinated peptide antibodies were 660.0 UI/mL and 560.0 U/ml, respectively. Serology for B19 parvovirus, HIV, viral hepatitis, and Epstein-Barr virus infection were negative. On abdominal ultrasonography, splenomegaly of 17.9 cm was identified. Bone marrow biopsy and peripheral blood flow cytometry showed no evidence of large granular lymphocytic leukemia. Skin and sputum culture showed the growth of multisensitive Pseudomonas aeruginosa, although blood cultures were negative. Additional studies were unremarkable. Based on these findings, the diagnosis of Felty syndrome (FS) complicated with ecthyma gangrenosum (EG) due to Pseudomonas aeruginosa was established. Piperacillin/tazobactam was started with complete healing of lesions. Prednisolone was increased to 0.5 mg/kg/day with normalization of the neutrophil count. One month later, methotrexate was started and prednisolone was tapered.

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