Gianotti Crosti Syndrome

Gianotti Crosti Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Gianotti Crosti Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

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

Disorder Subdivisions

  • None

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.

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

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For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

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

Resources

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For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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