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GIANOTTI-CROSTI SYNDROME

GIANOTTI-CROSTI SYNDROME
Synonym

  • Papular acrodermatitis of childhood

    Key features

  • Symmetric papular eruption of extremities, face, and buttocks
  • Epstein-Barr virus most common etiology
  • Association with hepatitis B virus in some patients

    Introduction and History

    Gianotti-Crosti syndrome, also termed 'papular acrodermatitis of childhood', was first described by Gianotti in 1955 and by Gianotti and Crosti in 1956. This syndrome is characterized by the acute onset of a symmetric papular eruption localized to the face, extremities, and buttocks, and it occurs primarily in children. In 1964, Gianotti and Crosti reported the coexistence of anicteric hepatitis and Gianotti-Crosti syndrome, and in the 1970s the potential association with hepatitis B virus was suggested by several groups. Subsequent reports demonstrated that Gianotti-Crosti syndrome can be associated with a variety of viral agents (Table 81.3). Epidemiology

    Gianotti-Crosti syndrome has a worldwide distribution, and occurs most often in young children. In one large series of patients, the age range was between 6 months and 14 years, with a mean age of 2 years26. The syndrome tends to occur most commonly in spring and early summer. Pathogenesis
    Table 81-3. Potential etiologies reported in association with Gianotti-Crosti syndrome. POTENTIAL ETIOLOGIES REPORTED IN ASSOCIATION WITH GIANOTTI-CROSTI SYNDROME
    Viral
  • Hepatitis B*
  • Epstein-Barr virus§
  • Hepatitis A and C virus
  • Cytomegalovirus
  • Coxsackie virus
  • Respiratory syncytial virus
  • Adenovirus
  • Parainfluenza virus
  • Rotavirus
  • Parvovirus B19
  • Mumps virus
  • Human herpesvirus-6
  • Human immunodeficiency virus

    Non-viral
  • Group A ß-hemolytic streptococci
  • Mycobacterium tuberculosis

    Vaccines
  • Polio
  • DPT
  • MMR

    * Reported most commonly in Europe. §Most common cause in United States. DPT, diphtheria-pertussis-tetanus; MMR, measles-mumps-rubella.
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    page 1265

    Figure 81.10 Gianotti-Crosti syndrome. Multiple, edematous erythematous papules of the extensor extremities.
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    Gianotti-Crosti syndrome is regarded as a self-limited cutaneous response to various infections. Hepatitis B virus and EBV are the most frequently reported etiologic agents, with EBV being by far the most common cause in the United States. Reported potential etiologic associations are summarized in Table 81.3. Although the pathogenic mechanisms at play in Gianotti-Crosti syndrome are not clear, there is some suggestion that immunizations or immune imbalance may enhance the risk of developing Gianotti-Crosti syndrome after certain infections.

    Clinical Features and Differential Diagnosis
    Gianotti-Crosti syndrome is often preceded by an upper respiratory syndrome with mild constitutional symptoms. The exanthem begins abruptly, with monomorphous, skin-colored to pink-red, edematous papules that are symmetrically distributed on the face, buttocks and extensor surface of the extremities (Fig. 81.10). The trunk is relatively spared. Lesions may occasionally be vesicular or purpuric and tend to be asymptomatic. Systemic manifestations include low-grade fever and lymphadenopathy, mainly inguinal and axillary, which can persist for a few months. Hepatomegaly and splenomegaly may occasionally be present.

    The differential diagnosis of Gianotti-Crosti syndrome includes drug eruption, papular urticaria, other viral exanthems, erythema multiforme and molluscum contagiosum. The lesional morphology and symmetric distribution of the eruption are helpful in narrowing the diagnosis. Laboratory studies are generally unnecessary and not useful, and evaluation for hepatitis should be performed only when there is clinical suspicion.
    The syndrome was traditionally divided into two clinical forms, the one associated with hepatitis, and the second associated with other etiologic factors. In 1992, a review of 308 cases in Italy failed to reveal any distinguishing clinical features between the two forms of the disease26.

    Pathology
    The histopathologic findings are non-specific and include epidermal acanthosis, hyperkeratosis and a lymphohistiocytic perivascular infiltrate in the dermis with dilatation of dermal capillaries.

    Treatment
    The treatment for Gianotti-Crosti syndrome is supportive. In a child with the acute onset of a symmetric papular eruption of the face, extremities and buttocks, a thorough history and physical examination should be performed. Evaluation for specific viral agents (e.g. hepatitis B virus) should be performed only if indicated. The prognosis is good with spontaneous resolution, usually within 3-4 weeks, although the process may occasionally persist for up to 8 weeks.

    (information from www.DermText.com)

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