Gianotti-crosti syndrome L44.4

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 31.12.2020

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Synonym(s)

Acrodermatitis infantile papules; Acrodermatitis papulosa eruptiva infantilis; Gianotti-Crosti syndromes; infantile papular acrodermatitis; Papular eruptions of infants

History
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Gianotti, 1967; Crosti, 1955

Definition
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Reactive, intermittent, symmetrical, papular exanthema in young children, which has been described primarily in association with HBV infection, but can also be observed in connection with numerous other viral diseases (e.g. EBV infection, Coxsackie virus infection).

Occurrence/Epidemiology
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Worldwide spread.

Etiopathogenesis
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The pathogenesis is largely unexplained.

Self-limited Id reactions to viral and bacterial antigens are discussed.

In larger studies, the connection with a viral infection was proven in about 50% of the patients (mostly EBV or hepatitis C/B; less frequently coxsackie viruses, cytomegaloviruses, adenoviruses, enteroviruses, RSV, parainfluenza virus, parvovirus B19, HHV-6, HHV-7, HIV, COVID-19). The occurrence of GCS in the context of an infection with Mollusca contagiosa is not entirely rare.

In industrialized countries, Gianotti-Crosti syndrome is most frequently associated with EBV.

In isolated cases, its occurrence has been associated with infections caused by β-hemolytic streptococci, Mycobacterium tuberculosis, or vaccinations against poliomyelitis, diphtheria, influenza, or pertussis.

Children with atopic diathesis are frequently affected.

Manifestation
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Occurs in children between 6 months and 14 years of age. The average age of first manifestation is about 2 years.

Localization
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Preferably face, here especially cheeks, extremities (extensor sides), buttocks (symmetrical).

Clinical features
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After a short prodromal phase with a slight feeling of illness, subfebrile temperatures, pharyngitis, gastrointestinal symptoms (nausea and diarrhoea), episodic, disseminated, red or brown, smooth or scaly, 0.1-0.5 cm large, clearly raised papules and vesicles appear, which can also confluence in places to form larger plaques (rather untypical).

The exanthema is typically not itchy.

A Köbner phenomenon (isomorphism) can often be detected (linear arrangement on externally irritated areas, e.g. in scratch marks).

Generalized lymphadenopathy (cervical, axillary, inguinal) and/or hepatomegaly (anicteric hepatitis) are possible.

Laboratory
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  • In 50-80% of cases: lymphocytosis, but also lymphopenia, eosinophilia, elevated ESR.
  • More rarely: serum transaminases elevation, positive hepatitis B or EBV serology, elevated IgE; evidence of type I sensitization.

Differential diagnosis
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General therapy
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Symptomatic.

External therapy
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Symptomatically with Lotio alba, 2% Polidocanol-Zinc shaking mixture R200 or 3%-Polidocanol ointment (e.g. Recessan) Glucocorticoids can generally be dispensed with.

Internal therapy
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For itching, oral antihistamines such as doxylaminosuccinate (for infants from 6 months of age, e.g. mereprine syrup 1-2 times 1 teaspoon/day).

Progression/forecast
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Favorable course. Exanthema and lymphadenopathy resolve spontaneously after 3-8 weeks. Any existing hepatitis generally takes a mild course. Only rarely transition to chronic aggressive hepatitis. Rare are post-inflammatory hyper- or hypopigmentations. Chronic or lethal courses have been described, but are absolute rarities.

Literature
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  1. Berger EM et al. (2012) Experience with molluscum contagiosum and associated inflammatory reactions in
    pediatric dermatology practice: the bump that rashes. Arch Dermatol 148):1257-1264.
  2. Brandt O et al (2006) Gianotti-Crosti syndrome. J Am Acad Dermatol 54: 136-145.
  3. Crosti A, Gianotti F (1967) Infantile papular acrodermatitis and lymphoreticulotropic viroses. Minerva Dermatol 42: 264-278
  4. Gianotti F (1955) Rilievi di una particolare casistica tossinfettiva caratterizzata de eruzione eritemato-infiltrativa desquamativa a focolai lenticolari, a sede elettiva acroesposta. G Ital Dermatol 96: 678-697
  5. Magyarlaki I et al (1991) Papular acrodermatitis of childhood (Gianotti-Crosti disease). Pedr Dermatol 8: 224-227
  6. Smith KJ, Skelton H (2000) Histopathologic features seen in Gianotti-Crosti syndrome secondary to Epstein-Barr virus. J Am Acad Dermatol 43: 1076-1079
  7. Haug S et al. (2002) Gianotti-Crosti syndrome following immunization Hautarzt 53: 683-685.
  8. Ricci G et al (2003) Gianotti-Crosti syndrome and allergic background. Acta Derm Venereol 83: 202-205
  9. Schopf, R. Gianotti-Crosti syndrome in Epstein-Barr virus infection. Dermatologist 46, 714-716 (1995).
  10. Stefanato CM et al (2000) Gianotti-Crosti syndrome presenting as lichenoid dermatitis. Am J Dermatopathol 22: 162-165
  11. Terasaki K et al (2003) Gianotti-Crosti syndrome associated with endogenous reactivation of Epstein-Barr virus. Dermatology 207: 68-71
  12. Velangi SS, Tidman MJ (1998) Gianotti-Crosti syndrome after measles, mumps and rubella vaccination. Br J Dermatol 139: 1122-1123

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Last updated on: 31.12.2020