POEMS syndrome is a rare disorder causing protein buildup in organs and tissue cells, peripheral nerve damage, and symptoms including peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. It is most common in older men in Japan and may be a rare blood disorder or immune system disorder.
POEMS syndrome refers to a rare disease that causes a buildup of proteins in organs and tissue cells in various parts of the body. This disorder also causes varying degrees of peripheral nerve damage. While POEMS syndrome can affect anyone at any age, it most commonly affects men over the age of 50.
This disorder is labeled POEMS syndrome as an acronym representing each of the symptoms related to this condition. The five main symptoms associated with this disorder are peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. While each of these is a common symptom of this rare condition, not all symptoms need to be present for a person to be diagnosed with this syndrome. A thorough examination of a patient presenting with some or all of these symptoms is necessary before a definitive diagnosis can be made.
The peripheral neuropathy common in this syndrome can affect multiple nerves at once. Organomegaly refers to enlarged organs, which are usually the spleen or liver and can affect both. Abnormal enlargement can also occur in the lymph nodes of people with this syndrome.
The endocrinopathy common in POEMS syndrome produces a change or disruption in a person’s hormone levels, commonly leading to a hormone imbalance. Endocrinopathy can also affect the sexual organs, preventing the ovaries or testicles from functioning properly. When monoclonal gammopathy occurs, it causes the production of immunoglobulins from bone marrow cells, which have grown out of control. Skin changes, which commonly occur in POEMS syndrome, include thickening of the skin and changes in pigmentation.
More specific symptoms of POEMS syndrome include fatigue, progressive weakness of the extremities, edema, neuropathy in the hands and feet, and vision changes. Many with this syndrome also have type 1 diabetes. While some people do not experience all of these symptoms, most people with this syndrome have symptoms of peripheral neuropathy and monoclonal gammopathy.
Although it is a very rare disease, cases of POEMS syndrome are found in various parts of the world. It is found more frequently, however, in older men in Japan, where it is also known as Crow-Fukase syndrome. Other names for POEMS syndrome used interchangeably are Takatsuki disease, PEP syndrome, and Shimpo syndrome. There is research suggesting that this syndrome may actually be a very rare blood disorder of unknown origin. Other research suggests that it falls under the classification of immune system disorders.
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