eMedicine Specialties > Pediatrics: General Medicine > Dermatology

Gianotti-Crosti Syndrome

Author: Howard Pride, MD, Associate Professor, Departments of Pediatrics and Dermatology, Geisinger Medical Center
Contributor Information and Disclosures

Updated: Sep 29, 2008

Introduction

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

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.

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.

Clinical

History

The rash of Gianotti-Crosti syndrome (GCS) usually has sudden onset and may be associated with an acute infectious illness or immunization. The rash is usually present for 2-4 weeks but can last as long as 4 months. It may be mildly pruritic.

Physical

  • Children with GCS generally appear healthy; however, the following may occur:
    • Multiple papules erupt on the face, buttocks, and extensor surface of the extremities; papules are symmetrically distributed, discrete, and flesh-toned–to–erythematous-to-brown and flat-topped.
    • The trunk is strikingly spared, although a transient eruption can occur.
    • Individual papules are monomorphous and range in size from 1-5 mm. They may be hemorrhagic or edematous to the point of forming vesicles. They may coalesce into larger plaques.
    • The face may be the only area of involvement. One report that highlighted this clinical presentation showed that all children with an exclusively facial eruption had Epstein-Barr virus.3
  • Hepatosplenomegaly and axillary or inguinal adenopathy are inconsistent findings.

Causes

More on Gianotti-Crosti Syndrome

Overview: Gianotti-Crosti Syndrome
Differential Diagnoses & Workup: Gianotti-Crosti Syndrome
Treatment & Medication: Gianotti-Crosti Syndrome
Follow-up: Gianotti-Crosti Syndrome
Multimedia: Gianotti-Crosti Syndrome
References
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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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Further Reading

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Keywords

Gianotti-Crosti syndrome, GCS, papular acrodermatitis of childhood, papulovesicular acrolocated syndrome, hepatitis B infection, hepatitis B, lymphadenopathy, rash Epstein-Barr virus, papular acrodermatitis, hepatosplenomegaly, inguinal adenopathy, rotavirus, cytomegalovirus, adenovirus, enterovirus, respiratory syncytial virus, parainfluenza virus, Parvovirus, paravaccinia, human herpesvirus 6, echovirus, molluscum contagiosum, human immunodeficiency virus, HIV, group A beta-hemolytic streptococci, Mycobacterium avium-intracellulare, Mycoplasma pneumoniae, Bartonella henselae, Borrelia burgdorferi, meningococcemia, polio, diphtheria, influenza, pertussis, measles, smallpox, hepatitis A

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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.

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of 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.

CME Editor

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.

 
 
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