$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.