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Disease Profile

Gianotti Crosti syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Acrodermatitis, infantile lichenoid; Acrodermatitis, papular infantile; Crosti-gianotti syndrome;

Summary

Gianotti Crosti syndrome (GCS) is a rare childhood skin condition characterized by a papular rash with blisters on the skin of the legs, buttocks, and arms. It typically affects children between 9 months and 9 years of age. Skin lesions typically last at least 10 days and often last for several weeks. The lesions are usually preceded by an underlying infection (usually a virus), which may cause associated symptoms such as low-grade fever, sore throat, or symptoms of an upper respiratory infection. When GCS is associated with hepatitis B, Epstein-Barr, or cytomegalovirus (CMV) infection, acute hepatitis may also occur. GCS is thought to be a hypersensitive response to the underlying infection. While in many countries the underlying cause is hepatitis B, this is rarely the cause in North America. GCS typically does not require treatment and goes away on its own within 1 to 3 months.[1][2] In some cases, a mild topical steroid cream may be prescribed to relieve itching.[3]

Cause

Gianotti Crosti syndrome (GCS) is thought to be due to a hypersensitive response to a previous infection. The underlying infection tends to correlate with the infectious agent endemic to a specific geographic region. For example, in Japan and Mediterranean countries, GCS is more commonly associated with hepatitis B virus infection. Since there is more universal use of hepatitis B immunization, Epstein-Barr virus is now the most common associated infection worldwide. GCS may also occur after coxsackievirus, infectious mononucleosis, cytomegalovirus, enterovirus infections, ECHO viruses, respiratory syncytial virus, or after vaccination with a live virus serum.[1][2][3] In most cases, no laboratory tests are needed when a person is diagnosed with GCS. However, if jaundice or an enlarged liver are present, testing for hepatitis B and elevated liver enzymes may be performed.[2]

The exact reasons for the cause and effect relationship between underlying infections and GCS are not well-understood. There does not appear to be any genetic or familial predisposition to developing GCS.[1][2]

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.

References

  1. Gianotti Crosti Syndrome. NORD. 2009; https://rarediseases.org/rare-diseases/gianotti-crosti-syndrome/.
  2. Sören A Craig-Müller. Gianotti-Crosti Syndrome. Medscape Reference. August 1, 2014; https://emedicine.medscape.com/article/911275-overview.
  3. Amanda Oakley. Infantile papular acrodermatitis. DermnetNZ. September 19, 2015; https://dermnetnz.org/viral/gianotti-crosti.html.

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