Gianotti-Crosti Syndrome and Hemangiomas

June 1, 2005

This 12-month-old infant comes to your office with an asymptomatic eruption of sudden onset that involves the arms and legs. The child has a mild fever.

Case 1:

This 12-month-old infant comes to your office with an asymptomatic eruption of sudden onset that involves the arms and legs. The child has a mild fever.

Will you need to biopsy this eruption to make your diagnosis?

Case 1: This child has Gianotti-Crosti syndrome--a condition first described by Dr Gianotti in 1955 as a specific manifestation of hepatitis B infection. Subsequent reports indicated that the condition could occur without associated hepatitis. The term papular acral dermatitis of childhood (PAC) was then used to describe hepatitis-associated eruptions, and papulovesicular acrolocalized syndrome (PALS) was used to describe the non-hepatitis eruptions. It has proved impossible to differentiate these 2 entities clinically. I think it is prudent to refer to these eruptions as Gianotti-Crosti syndrome--which encompasses all viral-induced papular and papulovesicular eruptions that are symmetrically distributed on acral sites.

The eruption is usually fully developed within 7 days, and its onset is often associated with the systemic symptoms of viral infection--if one is present. The lesions are monomorphic, dome-shaped, pink- to flesh-colored, 1- to 5-mm papules or papulovesicules that number in the hundreds; they occur on the arms and legs but usually spare the trunk. The eruption is surprisingly asymptomatic. The other surprising feature is that it can often take up to 8 weeks for the entire rash to disappear, which is usually long after the associated viral illness has resolved.

Most experts believe that this condition is an immunologic or inflammatory reaction to a viral infection. The histology is nonspecific, and the general and laboratory examinations show features of the associated viral illness. There is no specific treatment.

The importance of recognizing Gianotti-Crosti syndrome is to avoid unnecessary diagnostic tests and treatments. The rash is characteristic and resolves spontaneously, so it is best to leave it alone and to reassure the parents. The only time I perform hepatitis B investigations is when no other viral illness is present and the child has risk factors for hepatitis B virus infection.

Case 2:

This 3-month-old girl presented with a slowly growing "birthmark" on her upper eyelid that had started as a small "blush." The child was otherwise well.

How serious is this lesion? Would you reassure the parents that no treatment is needed--or will active intervention be required?

Case 2: Hemangiomas of infancy are benign neoplasms of capillaries that are initially proliferative; in most children, they involute spontaneously and regress completely.The proliferative phase occurs during the first 6 months of the child's life. The involution and regression proceed over the next 10 years at a rate of approximately 10% per year.

Hemangiomas of infancy may produce a number of complications. Anatomic location is of particular importance in determining risks associated with treatment. Periocular hemangiomas are particularly frightening to me because they may profoundly affect the eye and all treatments involve significant risk.

In this child, the hemangioma had begun to proliferate throughout the lid. Consequences include obstruction of light stimulation in the developing eye and pressure on the globe with resulting loss of vision in the affected eye.

Systemic corticosteroids are the treatment of choice for "significant" periocular hemangiomas. Doses of 2 to 3 mg/kg of prednisone are required--often for weeks to months. I have not found potent topical corticosteroids to be beneficial in any but the most insignificant periocular hemangiomas of infancy. Intralesional corticosteroids introduce the danger (albeit small) of particulate matter retinal embolization.

The response to systemic corticosteroids is often rapid. I always involve a pediatric ophthalmologist in the care of affected children and monitor them very closely--weekly or even twice weekly. Proliferating lesions are dangerous and can change overnight.