Dermatologic Manifestations of Gianotti-Crosti Syndrome 

  • Author: Kara N Shah, MD, PhD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jun 3, 2010
 

Background

Gianotti-Crosti syndrome is a self-limited childhood exanthem that manifests in a characteristic acral distribution. It is rarely associated with systemic findings. The original cases, described in Italy by Gianotti in 1955, were associated with hepatitis B virus infection, although other viral infections currently account for most cases.

The 2 older, descriptive designations, papular acrodermatitis of childhood (PAC) and papulovesicular acrolocated syndrome (PAS), described indistinguishable clinical entities. PAC is the term most commonly used today.

Also see the eMedicine Pediatrics article Gianotti-Crosti Syndrome.

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Pathophysiology

Although the original reports of Gianotti-Crosti syndrome (papular acrodermatitis of childhood) were attributed to acute infection with the hepatitis B virus, more recent studies have demonstrated that Gianotti-Crosti syndrome is more commonly associated with a number of other infectious agents, both viral and bacterial. In the United States, the agent that has been reported most frequently in association with Gianotti-Crosti syndrome is Epstein-Barr virus (EBV).

The pathophysiologic process underlying Gianotti-Crosti syndrome remains unknown, although it is believed to represent an immunologic response to transient viremia or bacteremia, possibly a delayed-type hypersensitivity response. Deposition of circulating immune complexes in the dermis may play a role. Several studies have failed to demonstrate deposition of viral particles or bacteria within the dermis.

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Epidemiology

Frequency

United States

Gianotti-Crosti syndrome (papular acrodermatitis of childhood) occurs sporadically in the clinical setting, with no apparent genetic or familial predisposition. Gianotti-Crosti syndrome appears to be uncommon and may go unrecognized because of its generally benign and self-limited course. Gianotti-Crosti syndrome is more commonly seen in the spring and summer, possibly as a result of a concomitant increase in viral illness seen in the general population.

International

The distribution of Gianotti-Crosti syndrome (papular acrodermatitis of childhood) is worldwide, with cases reported in Great Britain, France, Germany, Spain, Russia, Turkey, India, Hong Kong, China, and Japan. In one series of 20,000 patients younger than 5 years seen over a 5-year period in Bordeaux, France, 26 patients with features consistent with Gianotti-Crosti syndrome were identified, yielding an annual incidence of 0.13%.[1]

Mortality/Morbidity

Gianotti-Crosti syndrome (papular acrodermatitis of childhood) is generally a benign, self-limited condition.

In the original cases of hepatitis B virus-associated disease, anicteric hepatitis developed in a proportion of patients. Anicteric hepatitis may also be seen in cases associated with other viral illnesses such as EBV.

In extremely rare cases, chronic liver disease has followed the initial phase of infection with hepatitis B virus.

Race

No racial predilection is apparent for Gianotti-Crosti syndrome.

Sex

In children, males and females are equally affected. Reported cases in adults have been seen exclusively in females.

Age

The onset of the eruption typically occurs in children aged 3 months to 15 years, with an average age of 2 years and a peak incidence at 1-6 years. Adult cases are rare but have been reported in women aged 17-46 years.[2, 3, 4, 5, 6, 7]

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Contributor Information and Disclosures
Author

Kara N Shah, MD, PhD  Assistant Professor, Department of Pediatrics and Dermatology, University of Pennsylvania School of Medicine; Clinic Director, Pediatric Dermatology, Children's Hospital of Philadelphia

Kara N Shah, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Albert C Yan, MD  Section Chief, Associate Professor, Department of Pediatrics, Section of Dermatology, Children's Hospital of Philadelphia and University of Pennsylvania

Albert C Yan, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, Society for Investigative Dermatology, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: elsevier Royalty Other

Paul J Honig, MD  Director of Dermatology, Professor, Department of Pediatrics, Section of Pediatric Dermatology, Children's Hospital of Philadelphia, University of Pennsylvania

Paul J Honig, MD is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Dermatology, and American Academy of Pediatrics

Disclosure: Nothing to disclose.

Specialty Editor Board

Bernice R Krafchik, MBChB, FRCPC  Professor Emeritus, Department of Pediatrics, Section of Dermatology, University of Toronto

Bernice R Krafchik, MBChB, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, Canadian Medical Association, College of Physicians and Surgeons of Ontario, Royal College of Physicians and Surgeons of Canada, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Van Perry, MD  Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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Characteristic erythematous papules of Gianotti-Crosti syndrome appear on the face of this child. The child does not have a toxic appearance.
Characteristic erythematous papules of Gianotti-Crosti syndrome can be seen on the extremities, as is the case in this young child.
A 9-year-old girl who recently returned from a trip to Europe with her family. She developed a low-grade fever, malaise, and some lymphadenopathy. An eruption limited to her face, arms, legs, and buttocks was noted.
A mildly pruritic eruption characterized by erythematous papules localized to the face, arms, legs, and buttocks.
 
 
 
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