eMedicine Specialties > Dermatology > Pediatric Diseases

Gianotti-Crosti Syndrome (Papular Acrodermatitis of Childhood)

Author: Kara N Shah, MD, PhD, Assistant Professor, Department of Pediatrics, Section of Dermatology, Children's Hospital of Philadelphia
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; 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; Paul J Honig, MD, Director of Dermatology, Professor, Department of Pediatrics, Section of Pediatric Dermatology, Children's Hospital of Philadelphia, University of Pennsylvania
Contributor Information and Disclosures

Updated: Mar 24, 2008

Introduction

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.

Pathophysiology

Although the original reports of this syndrome 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). See

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.

Frequency

United States

Gianotti-Crosti syndrome occurs sporadically in the clinical setting, with no apparent genetic or familial predisposition. It appears to be uncommon and may go unrecognized because of its generally benign and self-limited course. It 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 the disease 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 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.

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.

Clinical

History

Children usually present with an acute, symmetric, exanthematous, asymptomatic cutaneous eruption that develops over several days.

  • The eruption typically lasts at least 10 days but can last longer than 6 weeks in more than 50% of patients. Complete resolution typically takes more than 2 months. Recurrences are rare.
  • Pruritus accompanies the eruption in 23% of patients.
  • Other symptoms related to the primary viral syndrome or underlying bacterial infection may include mild constitutional symptoms such as low-grade fever and malaise, pharyngitis and/or mucosal lesions, or symptoms of an upper respiratory tract infection.
  • When associated with a hepatitis B virus, EBV, or CMV infection, an anicteric acute hepatitis may be present.
  • A case control study of 29 children with Gianotti-Crosti syndrome from Bologna, Italy demonstrated a higher prevalence of atopic dermatitis in patients versus controls (24.1% vs 6.8%). They also found that patients were more likely than controls to have elevated levels of total and specific immunoglobulin E and a family history of atopy.2

Physical

The cutaneous eruption is characterized by monomorphous pale, pink-to-flesh–colored or erythematous 1- to 10-mm papules or papulovesicles localized symmetrically and acrally over the extensor surfaces of the extremities, the buttocks, and the face. The number of lesions ranges from few to many. The trunk, knees, elbows, palms, and soles are rarely involved, and, in general, extensive involvement of the trunk is not consistent with a diagnosis of Gianotti-Crosti syndrome. Individual papules may coalesce to form larger plaques. Uncommonly, the eruption may develop a petechial or purpuric appearance. Partial involvement of only the face or the extremities is not uncommon, especially in older children. Over days to weeks, the papules may acquire a smooth-topped, polished, or lichenoid appearance. Other findings upon physical examination include the following:

  • Fever (27%)
  • Lymphadenopathy (31%)
  • Hepatosplenomegaly (4%)
  • Pharyngeal erythema, oropharyngeal ulcers or vesicles, or tonsillar swelling in cases secondary to infections of the upper respiratory tract

Causes

Gianotti-Crosti syndrome has been associated with the following infectious agents:

  • Hepatitis B virus, most commonly ayw strain
  • Epstein-Barr virus (probably the most common etiology)3 : Gianotti-Crosti syndrome has been associated with both primary infection and with endogenous reactivation of EBV.4
  • Respiratory syncytial virus5
  • Coxsackieviruses,1,6 echoviruses, and other enteroviruses5
  • Parainfluenza virus
  • Parvovirus B197
  • Poxvirus7
  • Cytomegalovirus1
  • Human herpesvirus 6, both primary infection and reactivation of latent infection8
  • Rotavirus9
  • Human immunodeficiency virus
  • Group A beta-hemolytic streptococci
  • Neisseria meningitidis10
  • Mycoplasma pneumoniae
  • Bartonella henselae
  • Borrelia burgdorferi

Gianotti-Crosti syndrome has also been reported to occur after vaccination for the following:

  • Hepatitis A virus11
  • Hepatitis B virus12,13,14
  • Measles, mumps, rubella viruses (MMR)15
  • Influenza virus16
  • Oral poliovirus vaccine17
  • Japanese encephalitis virus18

The Medscape Vaccines Resource Center may be of interest. Additionally, see the CME course Promoting Vaccine Safety: Answers to Common Questions.

More on Gianotti-Crosti Syndrome (Papular Acrodermatitis of Childhood)

Overview: Gianotti-Crosti Syndrome (Papular Acrodermatitis of Childhood)
Differential Diagnoses & Workup: Gianotti-Crosti Syndrome (Papular Acrodermatitis of Childhood)
Treatment & Medication: Gianotti-Crosti Syndrome (Papular Acrodermatitis of Childhood)
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Multimedia: Gianotti-Crosti Syndrome (Papular Acrodermatitis of Childhood)
References
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References

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

  2. Ricci G, Patrizi A, Neri I, Specchia F, Tosti G, Masi M. Gianotti-Crosti syndrome and allergic background. Acta Derm Venereol. 2003;83(3):202-5. [Medline].

  3. Hofmann B, Schuppe HC, Adams O, et al. Gianotti-Crosti syndrome associated with Epstein-Barr virus infection. Pediatr Dermatol. Jul-Aug 1997;14(4):273-7. [Medline].

  4. Terasaki K, Koura S, Tachikura T, Kanzaki T. Gianotti-Crosti syndrome associated with endogenous reactivation of Epstein-Barr virus. Dermatology. 2003;207(1):68-71. [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. James WD, Odom RB, Hatch MH. Gianotti-Crosti-like eruption associated with coxsackievirus A-16 infection. J Am Acad Dermatol. May 1982;6(5):862-6. [Medline].

  7. Carrascosa JM, Just M, Ribera M, Ferrandiz C. Papular acrodermatitis of childhood related to poxvirus and parvovirus B19 infection. Cutis. May 1998;61(5):265-7. [Medline].

  8. Chuh AA, Chan HH, Chiu SS, Ng HY, Peiris JS. A prospective case control study of the association of Gianotti-Crosti syndrome with human herpesvirus 6 and human herpesvirus 7 infections. Pediatr Dermatol. Nov-Dec 2002;19(6):492-7. [Medline].

  9. Di Lernia V. Gianotti-Crosti syndrome related to rotavirus infection. Pediatr Dermatol. Nov-Dec 1998;15(6):485-6. [Medline].

  10. Khan I, Gleeson J, McKenna D. Gianotti-Crosti syndrome following meningococcal septicaemia. Ir Med J. Feb 2007;100(2):373. [Medline].

  11. Monastirli A, Varvarigou A, Pasmatzi E, Badavanis G, Georgiou S, Mantagos S, et al. Gianotti-Crosti syndrome after hepatitis A vaccination. Acta Derm Venereol. 2007;87(2):174-5. [Medline].

  12. Andiran N, Sentürk GB, Bükülmez G. Combined vaccination by measles and hepatitis B vaccines: a new cause of Gianotti-Crosti syndrome. Dermatology. 2002;204(1):75-6. [Medline].

  13. Karakas M, Durdu M, Tuncer I, Cevlik F. Gianotti-Crosti syndrome in a child following hepatitis B virus vaccination. J Dermatol. Feb 2007;34(2):117-20. [Medline].

  14. Tay YK. Gianotti-Crosti syndrome following immunization. Pediatr Dermatol. May-Jun 2001;18(3):262. [Medline].

  15. Velangi SS, Tidman MJ. Gianotti-Crosti syndrome after measles, mumps and rubella vaccination. Br J Dermatol. Dec 1998;139(6):1122-3. [Medline].

  16. Cambiaghi S, Scarabelli G, Pistritto G, Gelmetti C. Gianotti-Crosti syndrome in an adult after influenza virus vaccination. Dermatology. 1995;191(4):340-1. [Medline].

  17. Erkek E, Senturk GB, Ozkaya O, Bükülmez G. Gianotti-Crosti syndrome preceded by oral polio vaccine and followed by varicella infection. Pediatr Dermatol. Nov-Dec 2001;18(6):516-8. [Medline].

  18. Kang NG, Oh CW. Gianotti-Crosti syndrome following Japanese encephalitis vaccination. J Korean Med Sci. Jun 2003;18(3):459-61. [Medline].

  19. Boeck K, Mempel M, Schmidt T, Abeck D. Gianotti-Crosti syndrome: clinical, serologic, and therapeutic data from nine children. Cutis. Dec 1998;62(6):271-4; quiz 286. [Medline].

  20. Brandt O, Abeck D, Gianotti R, Burgdorf W. Gianotti-Crosti syndrome. J Am Acad Dermatol. 2006;Jan;54(1):136-45. [Medline].

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

  22. 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].

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

  24. de la Torre C. Gianotti-Crosti syndrome following milkers' nodules. Cutis. Nov 2004;74(5):316-8. [Medline].

  25. Hurwitz S. Papular acrodermatitis of childhood. In: Clinical Pediatric Dermatology. Philadelphia, Pa: WB Saunders; 1993:128-30.

  26. Patrizi A, Di Lernia V, Ricci G, Masi M, Varotti C. Papular and papulovesicular acrolocated eruptions and viral infections. Pediatr Dermatol. Mar 1990;7(1):22-6. [Medline].

  27. Schacher LA, Hansen R C. Gianotti-Crosti Syndrome. In: Pediatric Dermatology. Amsterdam: Elsevier; 2003:667-70.

  28. Yoshida M, Tsuda N, Morihata T, Sugino H, Iizuka T. 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].

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Further Reading

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Keywords

papulovesicular acrolocated syndrome, acropapulo-vesicular syndrome, infantile papular acrodermatitis, infantile lichenoid acrodermatitis, erythemato-papulous acrodermatitis, erythemato-vesiculo-papulous eruptive syndrome, acrodermatitis papulosa eruptiva infantilis, papular infantile acrodermatitis, acrodermatitis papulosa infantum, infantile eruptive papulous dermatitis, PAC, PAS

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Contributor Information and Disclosures

Author

Kara N Shah, MD, PhD, Assistant Professor, Department of Pediatrics, Section of 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; american college of physicians Honoraria 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.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

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.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

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; american college of physicians Honoraria Other

 
 
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