Published on 19/03/2015 by admin
Filed under Dermatology
Last modified 19/03/2015
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Carlo Gelmetti
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Gianotti–Crosti syndrome was described in 1955 by Ferdinando Gianotti as an exanthematic papular rash symmetrically distributed on the face, buttocks, and extremities. The disease had mild constitutional symptoms, was not preceded by fever or severe prodromes and had a benign course. The term ‘Gianotti–Crosti syndrome’ (GCS) is now used to include all eruptive acrolocated dermatoses characterized by papular or papulovesicular lesions caused by microorganisms or vaccines. The first association was with hepatitis B (HBV). In addition to viruses, GCS has been reported to occur after immunization with live, killed, and recombinant vaccines. As the number of immunizations increases and more combinations will be available, an increasing number of these post-immunization cases are expected.
Synonyms include papular acrodermatitis of childhood, infantile papular acrodermatitis, papulovesicular acrolocated syndrome, Crosti–Gianotti disease, Gianotti disease.
The diagnosis of GCS is clinical. Like other viral exanthems, it mainly affects children of preschool age, though adult cases have occurred. The typical eruption consists of monomorphic, lentil-sized lesions symmetrically distributed on the face, buttocks, and limbs. The lesions are papular or papulovesicular, sometimes edematous, and rarely purpuric. The trunk, antecubital, and popliteal surfaces are usually spared, even though a transient rash may be noted on the trunk in the early stage. Mucous membranes are not affected. The eruption develops within a week, typically beginning on the thighs and buttocks, then involving the extensor aspects of the arms, and finally the face. Individual lesions are a few millimeters in diameter (bigger lesions are uncommon), and vary in color from rose to red-brown. The pruritus is usually mild, and excoriation is rare. The lesions fade in 3 to 4 weeks with mild desquamation, and relapse is rare. A longer course of up to 6 to 8 weeks is occasionally seen. Inguinal and axillary lymphadenopathy is common.
In cases associated with HBV, the hepatitis begins at the same time as, or 1 to 2 weeks after, the onset of the GCS. The liver is usually enlarged but not tender; jaundice is exceptional. There are high levels of liver enzymes, and the viral markers become detectable in the serum depending on the duration of infection. Complications are rare, although some patients developed chronic periportal hepatitis. Abnormalities in the peripheral blood are inconsistent: there may be a leukopenia or a slight leukocytosis with 2–15% of monocytes; the erythrocyte sedimentation rate is not raised.
In HBV-negative cases, hepatomegaly and liver function abnormalities, if present, are mild, probably because some viruses are considered minor hepatitic viruses (e.g., Epstein–Barr virus, EBV).
There is no specific recommended treatment for GCS. However, when itching is disturbing, oral antihistamines or topical antipruritics can be used symptomatically. Although topical and systemic corticosteroids are sometimes used, the benefit is not established, since corticosteroids could prolong or delay the recovery of the disease.
In cases associated with HBV, the hepatitis can be treated with Interferon Alpha-2b or pegylated interferon with or without nucleoside analogs such as adefovir, entecavir, telbivudine, tenofovir, and lamivudine, the last approved also for children. It is important to investigate and treat other family members who may be carriers of the HBV or may benefit from vaccination. Vaccination against HBV, which will potentially eradicate all cases of GCS due to this virus, seems also effective in reducing the incidence of hepatocellular carcinoma. Other infective causes may also require treatment if identified.
Liver enzymes
Viral serology for Epstein–Barr virus, hepatitis B, hepatitis A, cytomegalovirus
Total IgE (PRIST) and specific IgE (RAST)
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
