Disease Information for Papular acrodermatitis/childhood

Gianotti Crosti Syndrome; Acrodermatitis, Infantile Lichenoid; Acrodermatitis, Papular Infantile; Crosti-Gianotti Syndrome; GCS; Papular Acrodermatitis of Childhood;

Gianotti-Crosti Syndrome is a rare skin disease affecting children between the ages of nine months to nine years;

Major symptoms may include blisters on the skin of the legs, buttocks and arms; The disorder is usually preceded by a viral infection---[NORD 2005]---------------------------Gianotti-Crosti syndrome, or papular acrodermatitis of childhood (PAC), is a self-limited childhood exanthem that manifests in a characteristic acral 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, although other viral infections currently account for most cases;

The 2 older, descriptive designations, PAC and papulovesicular acrolocated syndrome (PAS), described indistinguishable clinical entities; PAC is the term most commonly used today; Over the course of 3 or 4 days a profuse eruption of dull red spots develops first on the thighs and buttocks, then on the outer aspects of the arms, and finally on the face; The rash is often asymmetrical;

The individual spots are 5-10 mm in diameter and are a deep red colour; Later they often look purple, especially on the legs, due to leakage of blood from the capillaries; They may develop fluid-filled blisters; Itch is uncommon, particularly if hepatitis B is the cause-------------[dermnet nz website 2006]-----------

Pathophysiology: Although the original reports of this syndrome were attributed to acute infection with the hepatitis B virus, more recent studies have demonstrated that Gianotti-Crosti syndrome is more commonly associated with a number of other infectious agents; The agents that have been reported in association with Gianotti-Crosti syndrome include Epstein-Barr virus (EBV), cytomegalovirus (CMV), respiratory syncytial virus (RSV), coxsackievirus and other enteroviruses, parainfluenza virus, parvovirus B19, poxvirus, human herpesvirus v 6 (HHV-6), rotavirus, human immunodeficiency virus, and group A beta-hemolytic streptococci; Some occurrences have followed immunization with measles-mumps-rubella, hepatitis B, poliovirus, and influenza virus vaccines; Gianotti-Crosti syndrome likely represents a localized cutaneous inflammatory response to deposition of viral particles or bacteria within the dermis as a result of transient viremia or bacteremia; Deposition of immune complexes in the skin may also play a role;

(Edit)