Gianotti-Crosti Syndrome 

  • Author: Howard Pride, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 8, 2010
 

Background

Gianotti-Crosti syndrome (GCS) is a distinct infectious exanthem with associated lymphadenopathy and acute anicteric hepatitis.[1] Gianotti and Crosti initially described GCS as associated with a hepatitis B virus exanthem, which they termed papular acrodermatitis of childhood. A similar constellation of characteristics was later found to be associated with several infectious agents and immunizations that were called papulovesicular acrolocated syndromes. Subsequent retrospective studies have shown that these 2 entities are indistinguishable from one another, and they are now consolidated under the unifying title of GCS.[2]

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Pathophysiology

The most likely explanation for the exanthem is a local type IV hypersensitivity reaction to the offending viral or bacterial antigen within the dermis. This is based on the immunohistochemical characterization of the cutaneous inflammatory infiltrate. Findings on direct immunofluorescence examination of the skin are always negative. Electron microscopy has never revealed virus particles that suggested a reactive process other than an autoimmune phenomenon or direct infection of the skin. Inciting factors include various viral and bacterial infections, as well as recent immunizations. The rarity of GCS in adults suggests lifelong immunity to a common viral triggering agent. GCS is more common among children with atopic dermatitis, suggesting an immune mechanism. However, more information is needed in order to define the precise mechanism involved.

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Epidemiology

Frequency

United States

Because of the benign self-limited nature of GCS, most cases are not reported, and the overall incidence is unknown. Frequency probably parallels the incidence of a precipitating infection in a specific geographic region.

International

The underlying infection correlates with the endemic pathogens of a specific geographic region.

For example, in Japan and Mediterranean countries, GCS is more commonly associated with hepatitis B virus infection. With the advent of more universal hepatitis B immunization, Epstein-Barr virus is now the most common etiologic factor worldwide.

Mortality/Morbidity

  • The mere presence of a rash elicits some degree of social morbidity, depending on the age of the affected child.
  • Although typically nonpruritic, some reports document pruritus in the later stages of the rash.
  • The only significant morbidity involves the underlying infectious process, particularly the hepatitis B virus.

Race

No racial predilection has been noted; however, the underlying infection correlates with the endemic pathogens of a specific geographic region.

Sex

In the pediatric population, GCS affects males and females with equal frequency. However, affected adults have been exclusively female.

Age

GCS primarily occurs in children aged 3 months to 15 years, with a peak in children aged 1-6 years. More than 90% of patients are younger than 4 years.

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Contributor Information and Disclosures
Author

Howard Pride, MD  Associate Professor, Departments of Pediatrics and Dermatology, Geisinger Medical Center

Howard Pride, MD is a member of the following medical societies: American Academy of Dermatology and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Kevin P Connelly, DO  Clinical Assistant Professor, Department of Pediatrics, Division of General Pediatrics and Emergency Care, Virginia Commonwealth University; Medical Director, Paws for Health Pet Visitation Program of the Richmond SPCA; Pediatric Emergency Physician, Emergency Consultants Inc, Chippenham Medical Center

Kevin P Connelly, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Merrily P M Poth, MD  Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences

Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society

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

  1. Baleviciene G, Maciuleviciene R, Schwartz RA. Papular acrodermatitis of childhood: the Gianotti-Crosti syndrome. Cutis. Apr 2001;67(4):291-4. [Medline].

  2. Caputo R, Gelmetti C, Ermacora E, et al. Gianotti-Crosti syndrome: a retrospective analysis of 308 cases. J Am Acad Dermatol. Feb 1992;26(2 Pt 1):207-10. [Medline].

  3. Yoshida M, Tsuda N, Morihata T, et al. Five patients with localized facial eruptions associated with Gianotti-Crosti syndrome caused by primary Epstein-Barr virus infection. J Pediatr. Dec 2004;145(6):843-4. [Medline].

  4. Mendoza N, Diamantis M, Arora A, et al. Mucocutaneous manifestations of Epstein-Barr virus infection. Am J Clin Dermatol. 2008;9(5):295-305. [Medline].

  5. Draelos ZK, Hansen RC, James WD. Gianotti-Crosti syndrome associated with infections other than hepatitis B. JAMA. Nov 7 1986;256(17):2386-8. [Medline].

  6. Brandt O, Abeck D, Gianotti R, Burgdorf W. Gianotti-Crosti syndrome. J Am Acad Dermatol. 2006;54:136-145. [Medline].

  7. Chuh A, Lee A, Zawar V. The diagnostic criteria of Gianotti-Crosti syndrome: are they applicable to children in India?. Pediatr Dermatol. Sep-Oct 2004;21(5):542-7. [Medline].

  8. Chuh AA. Diagnostic criteria for Gianotti-Crosti syndrome: a prospective case-control study for validity assessment. Cutis. Sep 2001;68(3):207-13. [Medline].

  9. Magyarlaki M, Drobnitsch I, Schneider I. Papular acrodermatitis of childhood (Gianotti-Crosti disease). Pediatr Dermatol. Sep 1991;8(3):224-7. [Medline].

  10. Taieb A, Plantin P, Du Pasquier P, et al. Gianotti-Crosti syndrome: a study of 26 cases. Br J Dermatol. Jul 1986;115(1):49-59. [Medline].

 
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Multiple erythematous flat-topped papules on the cheeks of an 18-month-old boy with Gianotti-Crosti syndrome.
Arm of a 3-year-old boy with Gianotti-Crosti syndrome demonstrating well-defined erythematous lichenoid papules on the arm and forearm.
Thigh of the 3-year-old boy with Gianotti-Crosti syndrome.
 
 
 
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