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Gianotti Crosti Syndrome

Synonyms of Gianotti Crosti Syndrome

  • Acrodermatitis, Infantile Lichenoid
  • Acrodermatitis, Papular Infantile
  • Crosti-Gianotti Syndrome
  • GCS
  • PAC
  • Papular Acrodermatitis of Childhood
  • PAS

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Gianotti-Crosti Syndrome is a rare skin disease affecting children between the ages of nine months and 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 recur. 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 Populations

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.)

Standard Therapies

Because Gianotti-Crosti Syndrome is a self-limiting disorder, the treatment of affected children is primarily symptomatic and supportive. For example, in some cases, the use of topical ointments or certain medications by mouth may be recommended to help alleviate mild to potentially severe itching (pruritus). The skin lesions associated with Gianotti-Crosti Syndrome typically spontaneously resolve within approximately 15 to 60 days. When associated findings include enlargement of the lymph nodes (lymphadenopathy) and/or enlargement of the liver (i.e., in association with liver inflammation [hepatitis]), such findings may persist for several months after initial symptom onset.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Organizations related to Gianotti Crosti Syndrome

References

TEXTBOOKS
Nelson Textbook of Pediatrics, 15th Ed.: Richard E. Behrman, Editor; W.B. Saunders Company, 1996. P. 1868.

JOURNAL ARTICLES
The Gianotti-Crosti Syndrome. D. Rubenstein et al.; Pediatrics (March 1978; 61 (3)). Pp. 433-37.

Gianotti-Crosti Syndrome. A Review of Ten Cases Not Associated with Hepatitis-B. K.L. Spear et al.; Arch Dermatol (July 1984; 120 (7)). Pp. 891-96.

Gianotti-Crosti Syndrome: A Study of 26 Cases. A. Taieb et al.; Br J Dermatol (July 1986; 115, (1)). Pp. 49-59.

An Epidemic of Infantile Papular Acrodermatitis (Gianotti-Crosti Syndrome) Due to Epstein-Barr Virus. U. Baldari et al.; Dermatology (1994; 188(3)). Pp. 203-04.

Gianotti-Crosti Syndrome in Epstein-Barr Virus Infection. R.E. Schopf; Hautarzt (Oct 1995; 46(10)). Pp. 714-16.

Case Report: Gianotti-Crosti Syndrome Associated with Human Herpesvirus-6 Infection. S. Yasumoto et al.; J Dermatol (Jul 1996; 23(7)). Pp. 499-501.

Gianotti-Crosti Syndrome due to a Mixed Infection Produced by the Mumps Virus and the Parainfluenza Virus Type 2. R. Hergueta Lendinez et al.; An Esp Pediatr (Jan 1996; 44(1)). Pp. 65-66.

Gianotti-Crosti Syndrome Associated with Epstein-Barr Virus Infection. B. Hofmann et al.; Pediatr Dermatol (Jul-Aug 1997; 14(4)). Pp. 273-77.

Papular Acrodermatitis of Childhood Related to Poxvirus and Parvovirus B19 Infection. J.M. Carrascosa et al.; Cutis (May 1998; 61(5)). Pp. 265-67.

Gianotti-Crosti Syndrome: Clinical, Serologic, and Therapeutic Data from Nine Children. K. Boeck et al.; Cutis (Dec 1998; 62(6)). Pp. 271-74. Quiz: P. 286.

Gianotti-Crosti Syndrome Related To Rotavirus Infection (letter). V. Di Lernia; Pediatr Dermatol (Nov-Dec 1998; 15(6)). Pp. 485-86.

FROM THE INTERNET
eMedicine-Gianotti-Crosti Syndrome: Article by Kara N Shah, MD, Ph.D

hhtp://www.emedicine.com/derm/topic165.htm

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2009/04/10 00:00:00 GMT+0

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