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$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.