$Unique_ID{BRK03769} $Pretitle{} $Title{Gianotti-Crosti Syndrome} $Subject{Gianotti-Crosti Syndrome Acrodermatitis, Papular Infantile Crosti-Gianotti Syndrome Acrodermatitis, Infantile Lichenoid Hepatitis-B Virus Coxsackievirus Cytomegalovirus Infectious Mononucleosis} $Volume{} $Log{} Copyright (C) 1989 National Organization for Rare Disorders, Inc. 660: Gianotti-Crosti Syndrome ** IMPORTANT ** It is possible that the main title of the article (Gianotti-Crosti) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Acrodermatitis, Papular Infantile Crosti-Gianotti Syndrome Acrodermatitis, Infantile Lichenoid Information on the following diseases can be found in the Related Disorders section of this report: Hepatitis-B Virus Coxsackievirus Cytomegalovirus Infectious Mononucleosis 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. Gianotti-Crosti Syndrome is a rare skin disease affecting children between the ages of nine months to nine years. Major symptoms may include blisters on the skin of the legs, buttocks and arms. The disorder is usually preceded by a viral infection. Symptoms Gianotti-Crosti Syndrome is characterized by blisters on the skin that may or may not itch. They are usually found on the face, buttocks, arms or legs. The blisters consist of large, flat-topped, fluid filled sacks. They usually occur along with upper respiratory tract infection. The blisters usually last from twenty to twenty-five days; they do not usually reoccur. There may be an enlargement of the lymph nodes in the trunk area of the body. Gianotti-Crosti Syndrome usually occurs after a bout with a viral disease such as: Coxsackievirus, Hepatitis-B, Infectious Mononucleosis or Cytomegalovirus, or after vaccination with a live virus serum. Causes The cause of Gianotti-Crosti Syndrome is thought to be a reaction to a previous viral infection. In many countries the predisposing cause is usually the Hepatitis-B virus. In North America other viruses are more often the predisposing cause. The exact reasons for this cause and effect situation are unknown. Affected Population Gianotti-Crosti Syndrome usually affects children between the ages of nine months and nine years of age. It affects males and females in equal numbers. Although the disorder is regularly associated with Hepatitis-B infections in other countries, in North America it is rarely the cause. Related Disorders The following viral infections can cause Gianotti-Crosti. Hepatitis-B Virus (HBV) is one of three viral agents which causes inflammation of the liver known as "Hepatitis" or "diffuse hepatocellular inflammatory disease". Hepatitis-B is characterized by fever, nausea, vomiting, and yellow discoloration of the skin (jaundice). In its most serious form Hepatitis-B can become a chronic infection, or may cause liver cancer. The hepatitis-B virus can be passed from mother to unborn child, and is highly contagious through bodily fluids such as blood, semen and possibly saliva. It is often spread from person to person through intravenous drug use. (For more information on this disorder, choose "Hepatitis" as your search term in the Rare Disease Database). Coxsackievirus is characterized by infections that occur primarily during the summer. It affects mostly young children, especially boys, and includes fever, sore throat, vomiting, headache, respiratory signs and symptoms, diarrhea, abdominal pain, rash and earache. Cytomegalovirus Infection (CMV) can occur congenitally, postnatally or at any age. CMV ranges in severity from a silent infection without consequences, to a disease manifested by fever, hepatitis, and (in newborns) severe brain damage, stillbirth or perinatal death. In severe cases hemorrhaging, anemia, and liver damage can occur. In infants it may cause low birth weight, fever, hepatitis, blindness, deafness, or seizures. (For more information on this disorder, choose "Cytomegalovirus" as your search term in the Rare Disease Database). Infectious Mononucleosis is characterized by an incubation period of from thirty to fifty days in young adults, and a shorter time in children. The symptoms include feeling unwell for a few days, headache, fever and sore throat, with extreme fatigue. The glands in the neck, armpits and groin swell and the eyes get puffy. There may be tonsillitis, rash, loss of appetite, and sensitivity to light. Other organs in the body may be affected. The spleen and liver may become enlarged. This infection is caused by the Epstein-Barr virus. (For more information on this disorder, choose "Mono" as your search term in the Rare Disease Database.) Therapies: Standard Treatment of Gianotti-Crosti Syndrome is symptomatic and supportive. Symptoms disappear usually within twenty to twenty-five days without treatment. Therapies: Investigational This disease entry is based upon medical information available through October 1989. 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 Gianotti-Crosti Syndrome, 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 NIH/National Institute of Allergy and Infectious Disease 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5717 Centers for Disease Control (CDC) 1600 Clifton Road, NE Atlanta, GA 30333 (404) 639-3534 References THE GIANOTTI-CROSTI SYNDROME. D. Rubenstein, et al.; Pediatrics (March, 1978, issue 61 (3)). Pp. 433-437. GIANOTTI-CROSTI SYNDROME. A REVIEW OF TEN CASES NOT ASSOCIATED WITH HEPATITIS-B. K.L. Spear, et al.; Arch Dermatol (July, 1984, issue 120 (7)). Pp. 891-896. GIANOTTI-CROSTI SYNDROME; A STUDY OF 26 CASES. A. Taieb, et al.; Br J Dermatol (July, 1986, issue 115, (1)). Pp. 49-59.