Childhood exanthem with a characteristic distribution.
Cases often are associated with viral infections. Systemic features are
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
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.
Caputo R, Gelmetti C, Ermacora E: Gianotti-Crosti syndrome: A
retrospective analysis of 308 cases. J Am Acad Dermatol
Chuh AA, Chan HH, Chiu SS, ...