$Unique_ID{BRK04157} $Pretitle{} $Title{Purpura, Schonlein-Henoch; Allergic Purpura} $Subject{Purpura, Schonlein-Henoch Anaphylactoid Purpura Allergic Purpura Henoch-Shonlein Purpura Rheumatic Purpura Nonthrombocytopenic Idiopathic Purpura Peliosis Rheumatica Hemorrhagic Capillary Toxicosis Henoch's Purpura Shonlein's Purpura Scurvy Gardener-Diamond Syndrome Common Purpura} $Volume{} $Log{} Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc. 405: Purpura, Schonlein-Henoch Allergic Purpura ** IMPORTANT ** It is possible the main title of the article (Schonlein-Henoch Purpura) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names, disorder subdivisions, and related disorders covered by this article. Synonyms Anaphylactoid Purpura Allergic Purpura Henoch-Shonlein Purpura Rheumatic Purpura Nonthrombocytopenic Idiopathic Purpura Peliosis Rheumatica Hemorrhagic Capillary Toxicosis DISORDER SUBDIVISIONS Henoch's Purpura Shonlein's Purpura Information on the following diseases can be found in the Related Disorders section of this report: Scurvy Gardener-Diamond Syndrome Common Purpura 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. Shonlein-Henoch Purpura is one of a group of disorders characterized by purplish or brownish red discolorations on the skin. These spots may be large or small. Internal bleeding may occur in various areas of the body. This blood vessel disorder may affect the skin, joints, gastrointestinal system, kidneys, and in a very few cases the central nervous system. Little is known about the cause of this form of purpura although it may be an allergic reaction which more often occurs in children than in adults. Some cases of Shonlein-Henoch characterized by joint disease without gastrointestinal problems are termed Shonlein's Purpura. Another form characterized by acute abdominal symptoms but without joint disease is known as Henoch's Purpura. This disorder runs a limited course with a good prognosis in most cases. Symptoms The first symptom of Shonlein-Henoch Purpura is usually reddening of the skin, slight swelling and a rash (hives). These hives are associated with inflammation of small blood vessels and/or bleeding under the skin with a brown or reddish-purple appearance. The hives seem to appear on the buttocks and lower extremities in most cases but may spread and/or become more severe. Fever and a general feeling of discomfort or weakness may also be present. Blood and plasma may accumulate in the joints or abdomen producing acute local pain. Iron deficiency anemia may develop because of gastrointestinal bleeding. Other gastrointestinal disturbances such as vomiting or blood in the stool may also occur. Central nervous system complications of Shonlein-Henoch Purpura may include headaches, perceptual changes and/or seizures. Kidney inflammation or lesions, which occur in approximately ten percent of cases, may signify a more serious form of this type of purpura and can appear at any time during the course of this illness. Causes The exact cause of Shonlein-Henoch Purpura is unknown. Some medical researchers believe it may be an extreme allergic reaction to certain foods (such as chocolate, milk, eggs or beans), various drugs or insect bites in sensitive people. At times, infections of the upper respiratory tract or Rubella (German Measles) have preceded outbreaks of Shonlein-Henoch Purpura, but no definite link with viral infections has been proven. Affected Population Shonlein-Henoch Purpura is most commonly observed in children but can occur at any age. It appears to affect males slightly more often than females with a ratio of thirty-five males to twenty-five females in one study. Related Disorders The Purpuras are a group of disorders characterized by purplish or brownish red discolorations on the skin. These spots may be large or small and are caused by bleeding into the skin tissue where the spots appear. Symptoms of the following disorders can be similar to Shonlein-Henoch Purpura. Comparisons may be useful for a differential diagnosis: Common Purpura is the most prevalent type of purpura and is characterized by the unusual appearance of spots (black and blue marks) that signify easy bruising. This inherited condition occurs most frequently in women over 50 years of age, particularly those whose skin tissue has become thinner. In the absence of serious injury, unsightly bruises tend to appear rather than significant bleeding under the skin. Fragility of the blood vessels in affected individuals can lead to excessive bleeding following surgery or even minor injury. The blood vessel walls may also become thinner due to some types of illnesses, infection, hypothyroidism, and/or excessive exposure to certain drugs. No standard therapy has been found for treating common purpura although brief courses of corticosteroids may help reduce bleeding. Estrogen may be administered to affected women after menopause to help alleviate the tendency to bleed excessively. (To locate information on other types of purpura, or purpura as a symptom of other diseases, choose "purpura" as your search term in the Rare Disease Database). Scurvy is a type of purpura due to deficiency of ascorbic acid (Vitamin C) in the diet. It is marked by weakness, anemia, spongy gums, a tendency to bleed under the skin and in the mucous membranes. Adequate Vitamin C in the diet can cure this disorder. Gardener-Diamond Syndrome is a type of purpura occurring chiefly in young women. Spontaneous, painful, recurrent blue or purplish spots may appear on any part of the body unrelated to apparent injury. It has been suggested that this disorder may be triggered by the body's immune system attacking a specific component of blood cells. Gardner-Diamond is also called "Painful Bruising Syndrome". Therapies: Standard If a patient is found to be allergic to a substance which precipitated the attack of Shonlein-Henoch Purpura, that substance should be avoided. Other treatment is symptomatic and supportive. Mild cases in children tend to improve spontaneously with age. No specific therapy for cases with kidney involvement has been found, but hemodialysis may be of benefit in severe cases with renal failure. Therapies: Investigational Treatment with a combination of anticoagulants (heparin and acenocoumarol), corticosteroids, and immunosuppressive drugs has been tested for treatment of adults with severe cases of Shonlein-Henoch Purpura. Immunosuppressive drug therapy (cyclophosphamide) alone has been used in a few cases with some success. Plasmapheresis (a method for removing unwanted substances such as toxins, metabolic substances and plasma parts from the blood by separating plasma from blood cells has been tried. More research on the use of plasmapheresis as a treatment for Shonlein-Henoch Purpura is needed before effectiveness can be evaluated. This disease entry is based upon medical information available through February 1988. 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 Shonlein-Henoch Purpura, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 National Kidney Foundation 2 Park Avenue New York, NY 10016 (212) 889-2210 (800) 622-9010 American Kidney Fund 6110 Executive Blvd., Suite 1010 Rockville, MD 20852 (301) 881-3052 (800) 638-8299 (800) 492-8361 (MD) NIH/National Institute of Allergy and Infections Diseases (NIAID) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5717 NIH/National Heart, Lung and Blood Institute (NHLBI) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-4236 References SCHONLEIN-HENOCH SYNDROME IN ADULTS: D.A. Roth, et al.; Q J Med (May 1985, issue 55(217)). Pp. 145-152. CLINICAL ASPECTS OF THE NEPHROPATHY IN SCHONLEIN-HENOCH SYNDROME: E. Verrina, et al.; Pediatr Med Chir (May-June 1986, issue 8(3)). Pp. 317-320. NEUROLOGICAL MANIFESTATIONS OF SCHONLEIN-HENOCH PURPURA: REPORT OF THREE CASES AND REVIEW OF THE LITERATURE: A.L. Belman, et al.; Pediatrics (April 1985, issue 75(4)). Pp. 687-692.