$Unique_ID{BRK04114} $Pretitle{} $Title{POEMS Syndrome} $Subject{POEMS Syndrome Crow-Fukase Syndrome Shimpo's Syndrome Takatsuki's Syndrome PEP Syndrome Scleroderma Castleman's Disease} $Volume{} $Log{} Copyright (C) 19910 National Organization for Rare Disorders, Inc. 789: POEMS Syndrome ** IMPORTANT ** It is possible that the main title of the article (POEMS Syndrome) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Crow-Fukase Syndrome Shimpo's Syndrome Takatsuki's Syndrome PEP Syndrome Information on the following diseases can be found in the Related Disorders section of this report: Scleroderma Castleman's Disease General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. POEMS Syndrome is an acronym for Polyneuropathy (a disease of many nerves), Organomegaly (the enlargement of an organ), Endocrinopathy (a functional disorder of an endocrine gland), M protein (monoclonal immunoglobin, a type of antibody), and Skin changes. Symptoms Each letter of POEMS Syndrome stands for the following symptoms: Polyneuropathy is a disease of many nerves that causes tingling, numbness, burning pain, deficiencies in perception and vibratory sensations usually in the limbs. Organomegaly may affect any organ such as enlargement of the spleen (splenomegaly) or enlargement of the liver and the spleen (hepatosplenomegaly). Disease of the endocrine system may affect any endocrine gland (a gland that secretes hormones directly into the blood stream). Deficient secretion of the thyroid hormones (hypothyroidism) may occur. Sexual functioning may be affected in POEMS Syndrome due to dysfunction of hormone secreting glands. Adrenal secretion of hormones may also be insufficient. Elevated levels of M protein are usually found in the blood. Skin manifestations may include increased pigmentation (hyperpigmentation) and thickened skin resembling Scleroderma (see Related Disorders section of this report). Swelling of the lymph nodes (adenopathy) may also occur. An abnormal accumulation of fluid (edema) in the abdominal cavity (ascites), in connective tissue (anasarca), or in cells and tissues may also occur. Most of the patients have a malignant bone marrow disease called Multiple Myeloma. Abnormal proteins are usually present in the plasma. (For more information on this disorder, choose "Multiple Myeloma" as your search term in the Rare Disease Database.) Causes The exact cause of POEMS Syndrome is not known. It has been suggested that it may be an autoimmune disorder. Autoimmune disorders are caused when the body's natural defenses (antibodies, lymphocytes, etc.), against invading organisms suddenly begin to attack perfectly healthy tissue for unknown reasons. The abnormal proteins from the multiple myeloma tumor is another possible cause for this multi-system disease. Affected Population POEMS Syndrome is a rare disorder affecting males and females in equal numbers. Related Disorders Symptoms of the following disorders can be similar to those of POEMS Syndrome. Comparisons may be useful for a differential diagnosis: The skin changes of POEMS Syndrome are similar to Scleroderma. Scleroderma refers to a group of disorders characterized by the formation of fibrous tissue (fibrosis), degenerative changes, and vascular abnormalities in the skin. Scleroderma is the slow and continued (chronic) hardening and shrinking of the connective tissues of any part of the body. The skin may have firm violet patches with ivory/yellow centers, small chalk-white spots with or without a violet margin, tight glossy skin, or band-like strips of thickened skin. The internal organs may also be involved in some forms of Scleroderma. The exact cause is unknown. (For more information on this disorder, choose "Scleroderma" as your search term in the Rare Disease Database). The enlarged lymph nodes in POEMS Syndrome are very similar to Castleman's Disease. Castleman's Disease is a rare disorder of the lymphatic system characterized by noncancerous (benign) growths in lymph node tissue. The veins may be involved, causing a growth in the blood vessels themselves. Major symptoms may include development of a tumor, respiratory tract infections, coughing, lethargy, pressure or pain in the back and chest, difficulty in breathing, coughing up blood, and fever. The exact cause of Castleman's Disease is unknown, but it is thought to be an autoimmune disease. (For more information on this disorder, choose "Castleman's" as your search term in the Rare Disease Database). Therapies: Standard Treatment of POEMS Syndrome involves immunosuppressive drugs such as melphalan and prednisone. Therapies: Investigational Plasmapheresis may be of benefit in some cases of POEMS Syndrome. This procedure is a method for removing unwanted substances (toxins, metabolic substances and plasma parts such as antibodies and abnormal proteins) from the blood. Blood is removed from the patient and blood cells are separated from plasma. The patient's plasma is then replaced with other human plasma and the blood is retransfused into the patient. This therapy is still under investigation to analyze side effects and effectiveness. More research is needed before plasmapheresis can be recommended for use in all but the most severe cases of POEMS Syndrome. This disease entry is based upon medical information available through June 1991. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on POEMS Syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Arthritis, Musculoskeletal and Skin Diseases Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 References POEMS SYNDROME PRESENTING AS SYSTEMIC SCLEROSIS. CLINICAL AND PATHOLOGIC STUDY OF A CASE WITH MICROANGIOPATHIC GLOMERULAR LESIONS: J.P. Viard et al.; Am J Med (March, 1988, issue 84 (3 part 1). Pp. 524-8. THE SKIN CHANGES IN THE CROW-FUKASE (POEMS) SYNDROME. A CASE REPORT: W.B. & E.D. Shelley; Arch Dermatol (January, 1987, issue 123 (1)). Pp. 85-7.