Disease Information for Papular acrodermatitis/childhood

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Clinical Manifestations
Signs & Symptoms
Acrodermatitis/Rash Extremities and Face
Asymmetric rash/unilateral
Blebs/vesicles/skin
Blisters
Discreet Spots/dull red/blebs
Facial erythema
Facial rash
Foot Dermatitis
Foot rash
Lesions/rash extremities distribution
Non-pruritic rash
Papulosquamous Lesion
Papulosquamous rash
Rash
Rash hand/foot areas
Rash on extremities
Rash with well demarcated lesions
Rash/Characteristic pattern/distribution
Rash/upper arms
Vesicobullous rashes
Otherwise well Isolated problem
Clinical Presentation & Variations
Presentation/ Papular Rash Face Upper Arms
Disease Progression
Course/Short/brief/self-limited
Demographics & Risk Factors
Recent Event
Recent/Viral illness
Recent/Viral URI
Established Disease Population
Patient/Chronic active hepatitis
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Population/Preschool child
Laboratory Tests
Abnormal Lab Findings (Non Measured)
Right Shift (Viral) Differential Smear (Lab)
Associated Diseases & Rule outs
Associated Disease & Complications
Acrodermatitis, Papular, infantile
Papular acrodermatitis/childhood
Papular acrodermatitis/Hepatitis B syndrome
Viral exanthematous diseases
Disease Mechanism & Classification
Specific Agent
AGENT/Virus (category)
Pathophysiology
Pathophysiology/Post viral syndrome Multiple triggers
Process
PROCESS/Complicating disorder (ex)
PROCESS/Inflammatory/infection (ex)
Synonyms
Synonym
Acrodermatitides Infantile Papular, Acrodermatitis Infantile Papular, Acrodermatitis papulosa infantum, Giannotti Crosti, Gianotti Crosti Syndrome, Gianotti Crosti syndrome (disorder), Gianotti Crosti syndrome RETIRED, Infantile papular acroderm, Infantile Papular Acrodermatitides, infantile papular acrodermatitis, Infantile papular acrodermatitis (Giannotti Crosti), Pap acroderm of childhood, Papular Acrodermatitides Infantile, Papular Acrodermatitis Infantile, Papular acrodermatitis of childhood, Syndrome Gianotti Crosti, Synonym/GCS Hepatitis B exanthem, Synonym/Gianotti-Crosti syndrome
Definition

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;

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External Links Related to Papular acrodermatitis/childhood
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PubMed (National Library of Medicine)
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Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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