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At a glance

Gianotti-Crosti Syndrome (GCS) is a distinct infectious exanthem associated with lymphadenopathy and acute anicteric hepatitis. Cases often are associated with viral hepatitis B infections. Systemic features are rare.

Synonyms

Acrodermatitis Papulosa Infantum; Gianotti Disease.

History

The first case was described by C. Gianotti, an Italian pediatrician, in 1955. Historically, hepatitis B surface antigen-positive (HBsAg-positive) papular acrodermatitis of childhood (named Gianotti Disease) was first described and distinguished from HBsAg-Negative Papulovesicular, Acral Syndrome (called Gianotti-Crosti Syndrome), which was reported after contamination with different infectious agents (Ebstein-Barr virus, Coxsackie virus, Cytomegalovirus, Herpes virus 6, HIV, human Parvovirus B19). Therefore, all originally described patients were considered affected with hepatitis B virus infection. Nowadays other viral infections account for the majority of cases diagnosed with this medical condition.

Incidence

Affects children of both sexes, 80% of patients are between 2 and 6 years of age. Well known in Italy, where more than 300 cases were examined between 1955 and 1990. In the United States, because of the benign self-limited nature of Gianotti-Crosti Syndrome, 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. Internationally, the underlying infection correlates with the endemic pathogens of a specific geographic region. 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. However, worldwide distribution of the disease remains unknown.

Genetic inheritance

None. However, for an unknown reason, the incidence is higher in children with Down Syndrome.

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. Specific and self-limited response to cutaneous or mucosal contamination, opposite to the classic parenteral contamination. The disorder seems related to the presence of immune complexes in the vascular wall. Existence of immune cellular deficiency can explain the higher incidence of the disease in children with trisomy 21.

Diagnosis

Based on the clinical dermatological findings associated with a viral or streptococcal infection. GCS primarily occurs in children aged 3 months to 15 years, with a peak in children aged 1 to 6 years. More than 90% of patients are younger than 4 years, with a mean age of diagnosis of 15 months to 2 years. In the pediatric population, GCS affects males and females with equal frequency. However, affected adults have been almost exclusively female, with only three documented cases affecting men.

Clinical aspects

The affected children present with sometimes asymmetric, papulous or papulovesiculous, asymptomatic skin eruptions of up to 1 ...

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