POEMS syndrome (Osteosclerotic myeloma) is a very rare chronic multi-system paraneoplastic syndrome that is caused by abnormal plasma cells that produce excessive pro-inflammatory cytokines and angiogenic mediators. The acronym stands for Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes. The syndrome was first described by Crow (1956) and by Fukase (1968) leading to it sometimes being called Crow-Fukase syndrome.
The mean age of onset is 50 years of age and is almost twice as common in males.
Clinical Features:
Diagnosis is usually not made until 1-2 years after the first symptoms appear.
Common symptoms include progressive weakness of the nerves in the arms and legs (due to a sensorimotor polyneuropathy), hepatosplenomegaly, lymphadenitis, endocrinopathy (eg hypogonadism, adrenal insufficiency, diabetes mellitus, hypothyroidism), hyperpigmentation, hypertrichosis.
Diagnosis: Based on two mandatory criteria (polyneuropathy and plasma cell dyscrasia) and presence of one of three major criteria (Castleman’s disease, osteosclerotic lesions or elevated serum VEGF) and at least one of the six minor criteria (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilloedema or hematological changes).
Treatment:
Treatment is mostly symptomatic.
Options include radiation therapy (directed at the bone marrow), chemotherapy, hematopoietic cell transplantation or immunomodulatory drugs such as lenalidomide or thalidomide.
Relevance to Podiatry:
Sensory neuropathy putting the lower limb at risk for damage (eg foot ulcers); this may present as weakness, pain, numbness with a paresthesia in the toes and feet with a symmetric distribution that usually begins distally and progresses proximally.
Peripheral oedema is common.
Hyperhidrosis
Leukonychia and nail clubbing
Zhou et al (2024) presented a case report of a foot ulcer in which the patient had all the characteristics of it being diabetes. They were subsequently diagnosed with POEMS syndrome and the foot ulcer healed when the patient was treated with lenalidomide and dexamethasone.
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