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"My two-year-old son John has a very rare disease called Gianotti-Crosti (GCS).
Background: Gianotti-Crosti syndrome is a self-limited childhood exanthem that occurs in a characteristic distribution. It is rarely associated with systemic findings. The original cases, described in Italy by Gianotti in 1955, were associated with hepatitis B virus infection.

Pathophysiology: Following the original reports of this syndrome, cases in other countries have more commonly been associated with infectious agents other than hepatitis B; these agents include Epstein-Barr virus (EBV), respiratory syncytial virus (RSV), coxsackievirus and other enteroviruses, parainfluenza virus, parvovirus B19, poxvirus, cytomegalovirus (CMV), human herpesvirus v 6 (HHV-6), and group A beta-hemolytic streptococci. Some occurrences have followed immunization with measles-mumps-rubella, poliovirus, and influenza virus vaccines.

The 2 earlier designations, papular acrodermatitis of childhood (PAC) and papulovesicular acrolocated syndrome (PAS), described indistinguishable clinical entities. PAC is the term most commonly used.

Frequency:

    * In the US: Gianotti-Crosti syndrome sporadically occurs in the clinical setting, and it may go unrecognized because of its generally benign and self-limited course.

    * Internationally: One of the largest retrospective analyses of Gianotti-Crosti syndrome examined a total of 308 cases presenting to a clinic in Milan, Italy, during a period spanning 3 decades (1955-1989). The distribution of the disease is worldwide, with cases reported in Great Britain, France, Germany, Spain, Russia, and Japan.

     In one series of 20,000 patients younger than 5 years seen over a 5-year period in Bordeaux, France, 26 patients with features consistent with Gianotti-Crosti syndrome were identified, yielding an annual incidence of 0.13%.

Mortality/Morbidity:

    * Gianotti-Crosti syndrome is generally a benign, self-limited condition.

    * In extremely rare cases, chronic liver disease has followed the initial phase of infection with hepatitis B virus.

Sex: Males and females are equally affected.

Age:

    * The onset of the eruption typically occurs in persons aged 3 months to 7 years.

    * Adult cases are rare.

At the moment we are lucky that John is doing quite well. He was born a happy, healthy baby whom we discovered at around 12 months was deaf. His hearing over the next 6 months started to degenerate and then at around 18 months it was also discovered that John had some visual problems.