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Reminder of important clinical lesson
Case report
POEMS syndrome: a unique presentation and a diagnostic challenge
  1. Daniel Farrugia1,
  2. David James Camilleri1,
  3. Janice Azzopardi1 and
  4. Franco Camilleri2
  1. 1 Haematology, Mater Dei Hospital, Msida, Malta
  2. 2 Rheumatology, Mater Dei Hospital, Msida, Malta
  1. Correspondence to Dr Daniel Farrugia; daniel.f.farrugia{at}gov.mt

Abstract

We present the case of a previously well, 70-year-old lady who presented with gait deterioration and a clinicoradiological picture of myositis with T2-enhancement on MRI of the distal lower limb muscles. A muscle biopsy pointed towards neurogenic amyotrophy and a mixed demyelinating and axonal sensorimotor polyneuropathy was confirmed on nerve conduction studies. This was initially thought to be secondary to a myeloproliferative disorder but a positron emission tomography scan showed uptake in the left hip which corresponded to what was previously reported as a subchondral cyst on CT. A biopsy showed this to be a plasmacytoma in the context of a normal serum protein electrophoresis and a polyclonal increase in light chains on serum-free light chain estimation. The patient was started on treatment 6 months after the presentation for a diagnosis of POEMS syndrome, by which time, the patient had become bed-bound and fully dependent.

  • Muscle disease
  • Peripheral nerve disease
  • Musculoskeletal and joint disorders
  • Haematology (incl blood transfusion)
  • Radiology

https://doi.org/10.1136/bcr-2019-230284

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    Footnotes

    • Contributors All authors named, including the rheumatology and haematology specialists together with their trainees were involved in the diagnostic work up and care of the patient. The manuscript was drafted by DF and JA this was revised by DJC and FC.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Next of kin consent obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.