Gianotti-Crosti Syndrome

Childhood exanthem with a characteristic distribution. Cases often are associated with viral infections. Systemic features are rare.

Acrodermatitis Papulosa Infantum; Gianotti Disease.

First case was described by C. Gianotti, an Italian pediatrician, in 1955. All originally described patients were affected with hepatitis B virus infection (although nowadays other viral infections account for the majority of cases).

Affects children of both sexes, 80% of patients are between 2 and 6 years of age. Well known in Italy, where more than 300 cases were examined between 1955 and 1990. Worldwide distribution of the disease.

None, although the incidence is higher in children with Down syndrome.

Specific and self-limited response to cutaneous or mucosal contamination, opposite to the classic parenteral contamination. The disorder seems related to the presence of immune complexes in the vascular wall. Existence of immune cellular deficiency can explain the higher incidence of the disease in children with trisomy 21.

Based on the clinical dermatological findings associated with a viral or streptococcal infection.

Historically, hepatitis B surface antigen-positive (HBsAg-positive) papular acrodermatitis of childhood (named Gianotti Disease) was first described and distinguished from HBsAg-negative papulovesicular, acral syndrome (called Gianotti-Crosti Syndrome), which was reported after contamination with different infectious agents (Ebstein-Barr virus, Coxsackie virus, Cytomegalovirus, Herpes virus 6, HIV, human Parvovirus B19). The affected children present with sometimes asymmetric, papulous or papulovesiculous, asymptomatic skin eruptions of up to 1 cm in diameter that usually develop over 3 to 4 days, starting on the thighs or the buttocks and later spreading to the extensor surfaces of the extremities, and finally the face. The trunk is usually spared. Typically, these eruptions last at least 10 to 14 days, however in more than half of the patients the course is longer than 6 weeks. Secondary to leakage of blood from the capillaries, these lesions may turn into purple spots later in the course of the disease. Fluid-filled blisters may also occur. Pruritus is present only in about one quarter of the children and is particularly uncommon in patients where hepatitis B is responsible for the skin lesions. Other symptoms are mainly related to the underlying viral illness and may include malaise, a low-grade fever and other flulike symptoms. The classic form associated with hepatitis B (HBsAg-positive) typically presents with an acute, nonicteric hepatitis.

Assess liver function (e.g., transaminases, coagulation) and the degree of cytolysis in patients with underlying hepatitis. Exclude fever and respiratory tract infections. A complete blood count is recommended to assess transient lymphoblastosis, lymphocytosis, and thrombocytopenia.

Take the usual precautions for patients with hepatitis B.

Agents undergoing hepatic metabolism probably should be avoided.