Practice Essentials
Gianotti-Crosti syndrome (GCS) is a self-limited childhood exanthem that manifests in a characteristic acral distribution. It is rarely associated with systemic findings. Other descriptive designations, papular acrodermatitis of childhood (PAC) and papulovesicular acrolocated syndrome (PAS), described indistinguishable clinical entities. PAC is the term most commonly used today.
The original cases, described by Gianotti in 1955, were associated with hepatitis B virus infection, although other viral infections and vaccinations currently account for most cases. [1] 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. Children with a history of atopic dermatitis are at increased risk for developing GCS. [2]
The most common presentation is an acute, symmetric, exanthematous, asymptomatic cutaneous eruption that develops over several days, although pruritis is not uncommon. 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. Pruritus accompanies the eruption in 23% of patients. Systemic findings related to the primary viral syndrome or underlying bacterial infection may include malaise, fever, diarrhea, lymphadenopathy, and upper respiratory symptoms. [3]
Recurrences are rare, although a recurrent case associated with influenza virus vaccination has been reported. [4] Chronic cases lasting more than 12 months have been reported. [3]
Diagnostic criteria for Gianotti-Crosti syndrome have been proposed. All of the following positive clinical features must be present [5, 6] :
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Monomorphous, flat-topped, pink-brown papules or papulovesicles 1-10 mm in diameter
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At least three of the following four sites involved: cheeks, buttocks, extensor surfaces of forearms, extensor surfaces of legs
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Symmetric eruption
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Duration of at least 10 days
In addition, extensive truncal lesions and scaling are absent, alternative diagnoses based on clinical judgment have been excluded, and histopathologic findings are compatible, if skin biopsy is performed.
Treatment is primarily supportive. Topical steroids are generally not effective, although anecdotal responses have been reported. Systemic treatment with antihistamines has been moderately helpful in relieving pruritus. Some children may require symptomatic care for the associated viral or bacterial infection. Follow-up appointments are required to monitor the lesions and address parental concerns.
Discussion with the parents regarding the benign, self-limited course is advisable. If a particular viral or bacterial infection is suspected as the etiology, the course of the associated infection should also be discussed.
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. [2] 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.
Etiology
Gianotti-Crosti syndrome has been associated with the following infectious agents:
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Hepatitis B virus, most commonly ayw strain [7]
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Influenza virus, type A [19]
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Human herpesvirus 6, both primary infection and reactivation of latent infection [20]
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Herpes simplex virus-1 [21]
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Rotavirus [22]
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Hepatitis C virus [17]
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Human immunodeficiency virus [17]
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Group A beta-hemolytic streptococci
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Neisseria meningitidis [23]
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Mycoplasma pneumoniae [24]
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Bartonella henselae
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Borrelia burgdorferi
Gianotti-Crosti syndrome has also been reported to occur after vaccination for the following:
Epidemiology
United States and international statistics
In the United States, Gianotti-Crosti syndrome (papular acrodermatitis of childhood) 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. 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.
The distribution of Gianotti-Crosti syndrome 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%. [14]
Age-, sex-, and race-related demographics
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. [3] Adult cases are rare but have been reported mainly in women aged 17-65 years. [24, 42, 43, 44, 33, 45, 46, 47] Cases in immunocompromised adults with HIV-infection or following bone marrow transplantation have also been reported. [18]
In children, males and females are equally affected. Reported cases in adults have been seen almost exclusively in females.
No racial predilection is apparent for Gianotti-Crosti syndrome.
Prognosis
The prognosis is excellent. This syndrome is generally a benign, self-limited condition. The eruption usually starts to resolve after 6-8 weeks.
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.
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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.
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Characteristic erythematous papules of Gianotti-Crosti syndrome can be seen on the extremities, as is the case in this young child.
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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.
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A mildly pruritic eruption characterized by erythematous papules localized to the face, arms, legs, and buttocks.
