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Gianotti–Crosti syndrome


Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports

Management Strategy

In its classical clinical presentation, Gianotti–Crosti syndrome (GCS) is not difficult to diagnose with a presentation of a young child with symmetrical, erythematous, monomorphic papules, or vesicles, on the distal extensor extremities, and sometimes on the face and gluteal areas. Itching is usually slight to moderate. Subsidence within 2–6 weeks is the rule. Recurrences are exceptional. Regional lymphadenopathy is not uncommon. Non-icteric hepatitis may be associated in hepatitis B virus (HBV)-related GCS.

Atopic children are more predisposed to acquiring GCS. A prodrome involving the respiratory tract or gastrointestinal tract may be present.

HBV was associated with the earliest reported cases of GCS, especially in Italy and other European countries. However, Epstein–Barr virus (EBV) is now the predominant association worldwide. Many other viruses have also been implicated, including hepatitis A virus (HAV), hepatitis C virus, cytomegalovirus (CMV), Coxsackievirus, adenovirus, enterovirus, human herpesvirus-6B (HHV-6B), herpes simplex viruses, reovirus, varicella, roseola, rotavirus, respiratory syncytial virus, mumps, measles, parainfluenza, molluscum contagiosum, and human immunodeficiency virus (HIV). Non-viral microbial agents have also been implicated including Borrelia burgdorferi , Mycoplasma pneumoniae, Mycobacterium avium intracellulare , beta-hemolytic streptococci , meningococci , and Bartonella henselae . GCS has also been recognized following vaccinations, including oral poliomyelitis vaccine, and vaccines against diphtheria, pertussis, measles, HBV, and H1N1 influenza.

Abnormal liver enzymes and viral parameters may be present during active systemic infection involving the liver. However, chronic hepatitis is extremely rare.

GCS is typically a self-limiting disorder. Treatment is meant for symptomatic patients, consisting of oral antihistamines and topical soothing lotions like calamine. Emollients help children with an atopic background. Topical corticosteroids may be utilized for faster resolution of persistent inflammatory lesions in highly symptomatic patients. Systemic antibiotics may be required should pruritic lesions become secondarily infected.

Antiviral treatment has no role in managing GCS, as it does not resolve the dermatitis associated with proven active systemic viral disease.

Recurrences are exceptional. The long-term prognosis is good, with the possible exception of GCS patients associated with chronic active hepatitis due to HBV. Such patients should be assessed by expert hepatologists.

Diagnostic Criteria

A set of clinical diagnostic criteria has been validated in two independent studies, including positive and negative clinical features. Patients should exhibit all positive clinical features on at least one occasion or clinical encounter, and must not exhibit any negative clinical feature on any occasion or clinical encounter related to the rash. Additionally, no differential diagnosis should be considered more likely than GCS based on clinical judgment. If a lesional biopsy is performed, findings should be consistent with GCS.

Positive clinical features include:

  • Symmetrical rash

  • Monomorphous, flat-topped, pink-brown papules or papulovesicles, 1–10 mm in diameter

  • At least three of the following four sites involved: (i) cheeks; (ii) buttocks; (iii) extensor surfaces of forearms; and (iv) extensor surfaces of legs

  • Lasting for at least 10 days

Negative clinical features include:

  • Extensive truncal lesions

  • Scaly lesions

    Box 91.1
    Common Differential Diagnoses of Gianotti–Crosti syndrome

    • 1.

      Atopic dermatitis

    • 2.

      Papular urticaria

    • 3.

      Scabies

    • 4.

      Insect bite reaction

    • 5.

      Lichenoid eruptions

    • 6.

      Erythema multiforme

    • 7.

      Drug rash

    • 8.

      Henoch–Schönlein purpura

    • 9.

      Acrodermatitis enteropathica

Specific Investigations

The diagnosis of GCS is usually based on clinical criteria alone. Laboratory investigations are characteristically not required unless the patient’s clinical features suggest other possibilities as noted in the differential diagnosis of GCS.

  • Liver enzymes

  • Viral serologies: Epstein–Barr virus, hepatitis B, hepatitis A, hepatitis C, cytomegalovirus

Gianotti–Crosti syndrome

Brandt O, Abeck D, Gianotti R, et al. J Am Acad Dermatol 2006; 54: 136–45.

EBV is now the most common cause of GCS. Other viruses have been associated with GCS, including HAV; CMV; HHV-6B; Coxsackie A16, B4, and B5; rotavirus; parvovirus B19, respiratory syncytial virus, mumps virus, and parainfluenza virus types 1 and 2. HIV also appears to be capable of triggering GCS. The associations between various immunizations and GCS have long been known. Despite the proven connection between HBV and GCS, immunization against HBV only rarely causes GCS.

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