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$Unique_ID{BRK04159}
$Pretitle{}
$Title{Pyoderma Gangrenosum}
$Subject{Pyoderma Gangrenosum Leg Ulcers Dermatitis Herpetiformis Cutaneous
Sporotrichosis Ulcerative Colitis Crohn's Disease Rheumatoid Arthritis
Myelogenous Leukemia Myeloid Metaplasia Paraproteinemias}
$Volume{}
$Log{}
Copyright (C) 1988, 1989, 1992 National Organization for Rare Disorders,
Inc.
569:
Pyoderma Gangrenosum
** IMPORTANT **
It is possible that the main title of the article (Pyoderma Gangrenosum)
is not the name you expected. Please check the synonym list to find the
alternate names and disorder subdivisions covered by this article.
Synonyms
Leg Ulcers
Information on the following diseases can be found in the Related
Disorders section of this report:
Dermatitis Herpetiformis
Cutaneous Sporotrichosis
Ulcerative Colitis
Crohn's Disease
Rheumatoid Arthritis
Myelogenous Leukemia
Myeloid Metaplasia
Paraproteinemias
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.
Pyoderma Gangrenosum is a rare skin disorder of unknown origin. Major
symptoms include small pustules that develop into large ulcers at various
sites on the body. It may or may not be associated with other illnesses.
Symptoms
Pyoderma Gangrenosum is a skin ulceration characterized by a growing, purple
colored, undermined border with an irregular base of pus and decaying tissue.
The ulcers most frequently develop on the legs but they may appear on the
trunk, head and neck, scrotum and in the mucous membranes (mucosa).
Causes
The exact cause of Pyoderma Gangrenosum is not known although it is suspected
to be an autoimmune disease. (Autoimmune disorders are caused when the
body's natural defenses (antibodies) against invading organisms begin to
attack healthy tissue.) Fifty percent of all cases of Pyoderma Gangrenosum
develop for no apparent reason and the other fifty percent are associated
with other disorders. Of the associated diseases thirty percent are related
to ulcerative colitis (a digestive disease) but Pyoderma Gangrenosum is also
seen in patients with Crohn's disease, rheumatoid arthritis, acute and
chronic myelogenous leukemia, myeloid metaplasia and paraproteinemias.
Pyoderma Gangrenosum usually follows the course of the accompanying bowel
disease; however, it may appear during periods of disease remission as well.
Affected Population
Pyoderma Gangrenosum affects males and females in equal numbers. It is
uncommon in children and most common in middle-aged women.
Related Disorders
Symptoms of the following disorders can be similar to those of Pyoderma
Gangrenosum. Comparisons may be useful for a differential diagnosis:
Dermatitis Herpetiformis (Duhring Disease) is a familial disease
characterized by a chronic eruption of clusters of intensely itchy blisters,
papules, and slightly elevated patches on the skin that are either redder or
paler than the surrounding skin. Patches are usually distributed
symmetrically on elbows, knees, buttocks, head, and tail-bone area (sacrum).
Blisters and papules are common on the face and neck. Onset of Duhring
Disease can occur at any age, but it usually appears during middle adult
life. It is very rare in children, and occurs more frequently in males that
in females. (For more information on this disorder, choose "Duhring" as your
search term in the Rare Disease Database).
Cutaneous Sporotrichosis (Schenck Disease) is a chronic yeast infection
under the skin (subcutaneous) spread by way of the lymph glands and caused by
the bacteria known as Sporothrix Schenckii. The disease may remain localized
or may become generalized, involving bones, joints, lungs, and the central
nervous system. Lesions may be grainy, full of pus, ulcerative or draining.
The following disorders may precede the development of Pyoderma
Gangrenosum. They can be useful in identifying an underlying cause of some
forms of this disorder:
Ulcerative Colitis is a non-specific inflammatory disease of the bowel
characterized by chronic ulceration. The chief characteristic of this
disorder is bloody diarrhea. This disease is of unknown cause. It generally
begins in the area of the rectum. It may involve the entire large bowel.
The disease is usually chronic, with acute inflammation of the colon. It is
characterized by multiple, irregular superficial ulcerations, thickening of
the wall of the colon with scar tissue and polyps. (For more information on
this disorder, choose "Ulcerative Colitis" as your search term in the Rare
Disease Database).
Crohn's Disease is a form of inflammatory bowel disease, characterized by
severe chronic inflammation of the wall of the small intestine, but it can
involve any part of the gastrointestinal tract. The symptoms include
fatigue, anorexia, weight loss, abdominal pain, and chronic diarrhea. Less
commonly, there is inflammation of the mucosa of the mouth, the esophagus, or
stomach. Regional lymph nodes can become involved. A solid mass may be felt
in the abdomen during acute stages of the disease. (For more information on
this disorder, choose "Crohn" as your search term in the Rare Disease
Database).
Rheumatoid Arthritis is a disease of unknown origin which may have a
relationship to autoimmune processes. This disorder is characterized by lack
of appetite (anorexia), tiredness, painful and deformed joints, early morning
stiffness chiefly in the hands, knees, feet, jaw, and spine. Once affected,
a patient's joints remain painful or uncomfortable for weeks, months, or even
years. (For more information about many types of Arthritis use "Arthritis"
as your search term in the Rare Disease Database).
Myelogenous Leukemia is a form of blood cancer in which the abnormal
cells are derived from bone marrow (myelopoietic tissue).
Myeloid Metaplasia is a syndrome characterized by anemia, enlargement of
the spleen, nucleated red blood cells and immature granulocytes in the
circulating blood. If it occurs in persons who have another disease and it
is termed secondary or symptomatic myeloid metaplasia. It also occurs as a
primary illness and is then termed primary or agnogenic myeloid metaplasia,
myelofibrosis or myelosclerosis, because of the presence of an associated
fibrosis of the bone marrow. The condition may develop in the course of red
blood cell disease such as polycythemia rubra vera and there is a high
incidence of eventual development of myeloid leukemia.
Paraproteinemia is a disorder in which there is the presence of abnormal
proteins in the blood.
Therapies: Standard
Treatment of Pyoderma Gangrenosum consists of open wet dressings on the
ulcers, topical application of disodium cromoglycate or zinc sulfate, and
cleaning away the dead tissue. The skin must be protected from any other
injury which could result in development of other ulcers. In some cases, the
grafting of new skin to the wound may be recommended. Systemic treatment
includes the use of drugs such as corticosteroids, sulfonamides, sulfones and
antimetabolites, methylprednisolone, and dapsone.
Therapies: Investigational
The orphan drug thalidomide is being tested as a treatment for Pyroderma
Gangrenosum. This drug should not be taken by pregnant women because it can
cause severe birth defects. Physicians wishing to test thalidomide as a
treatment for this disorder may contact:
Pediatric Pharmaceutical
379 Thornall St.
Edison, NJ 08837
Thalidomide is available in England under special license from Penn
Pharmaceuticals of South Tredegar, South Wales.
Clinical trials are underway to study clofazimine in the treatment of
Pyroderma Gangreosum and other inflammatory diseases. Interested persons may
wish to contact:
Dr. Martin Carter
The Rockefeller University Hospital
Laboratory for Investigative Dermatology
New York, NY 10021
(212) 570-8091
to see if further patients are needed for this research.
This disease entry is based upon medical information available through
January 1992. 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 Pyroderma Gangrenosum, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
The National Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse
Box AMS
Bethesda, MD 20892
(301) 495-4484
For information about Colitis or Crohn's disease:
National Foundation for Ileitis and Colitis
444 Park Avenue, South
New York, NY 10016
(212) 685-3440
References
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and
Co., 1987. Pp. 1390-1392.
PYODERMA GANGRENOSUM ASSOCIATED WITH ULCERATIVE COLITIS: TREATMENT WITH
DISODIUM CROMOGLYCATE. D.R. Cave, et al.; Am J Gastroenterol (August, 1987,
issue 82 (8)). Pp. 802-804.
PYODERMA GANGRENOSUM COMPLICATING ULCERATIVE COLITIS: SUCCESSFUL
TREATMENT WITH METHYLPREDNISOLONE PULSE THERAPY AND DAPSONE. E. Galun, et
al.; Am J Gastroenterol (October, 1986, issue 81 (10)). Pp. 988-989.
PUSTULAR PYODERMA GANGRENOSUM ASSOCIATED WITH ULCERATIVE COLITIS IN
CHILDHOOD. REPORT OF TWO CASES AND REVIEW OF THE LITERATURE. L. Barnes, et
al.; J Am Acad Dermatol (October, 1986, issue 15 (4 Pt 1)). Pp. 608-614.