You are in: eMedicine Specialties > Pediatrics: General Medicine > Dermatology Gianotti-Crosti SyndromeArticle Last Updated: Dec 7, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 11
  Author: Howard Pride, MD, Associate Professor, Departments of Pediatrics and Dermatology, Geisinger Medical Center Howard Pride is a member of the following medical societies: American Academy of Dermatology and Society for Pediatric Dermatology Editors: Kevin P Connelly, DO, Clinical Assistant Professor, Department of Pediatrics, Division of General Pediatrics and Emergency Care, Virginia Commonwealth University; Medical Director, Paws for Health Pet Visitation Program of the Richmond SPCA; Pediatric Emergency Physician, Emergency Consultants Inc, Chippenham Medical Center; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School; Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center Author and Editor Disclosure Synonyms and related keywords: Gianotti-Crosti syndrome, GCS, papular acrodermatitis of childhood, papulovesicular acrolocated syndrome, hepatitis B infection, hepatitis B, lymphadenopathy, rash Epstein-Barr virus INTRODUCTIONSection 2 of 11
  BackgroundGianotti-Crosti syndrome (GCS) is a distinct infectious exanthem with associated lymphadenopathy and acute anicteric hepatitis. Gianotti and Crosti initially described GCS as associated with a hepatitis B virus exanthem, which they termed papular acrodermatitis of childhood. A similar constellation of characteristics was later found to be associated with several infectious agents and immunizations that were called papulovesicular acrolocated syndromes. Subsequent retrospective studies have shown that these 2 entities are indistinguishable from one another, and they are now consolidated under the unifying title of GCS. PathophysiologyThe most likely explanation for the exanthem is a local type IV hypersensitivity reaction to the offending viral or bacterial antigen within the dermis. This is based on the immunohistochemical characterization of the cutaneous inflammatory infiltrate. Findings on direct immunofluorescence examination of the skin are always negative. Electron microscopy has never revealed virus particles that suggested a reactive process other than an autoimmune phenomenon or direct infection of the skin. Inciting factors include various viral and bacterial infections, as well as recent immunizations. The rarity of GCS in adults suggests lifelong immunity to a common viral triggering agent. GCS is more common among children with atopic dermatitis, suggesting an immune mechanism. However, more information is needed in order to define the precise mechanism involved. FrequencyUnited StatesBecause of the benign self-limited nature of GCS, most cases are not reported, and the overall incidence is unknown. Frequency probably parallels the incidence of a precipitating infection in a specific geographic region. InternationalThe underlying infection correlates with the endemic pathogens of a specific geographic region. For example, in Japan and Mediterranean countries, GCS is more commonly associated with hepatitis B virus infection. With the advent of more universal hepatitis B immunization, Epstein-Barr virus is now the most common etiologic factor worldwide. Mortality/Morbidity
RaceNo racial predilection has been noted; however, the underlying infection correlates with the endemic pathogens of a specific geographic region. SexIn the pediatric population, GCS affects males and females with equal frequency. However, affected adults have been exclusively female. AgeGCS primarily occurs in children aged 3 months to 15 years, with a peak in children aged 1-6 years. More than 90% of patients are younger than 4 years. CLINICALSection 3 of 11
HistoryThe rash of Gianotti-Crosti syndrome (GCS) is usually of sudden onset and may be associated with an acute infectious illness or immunization. The rash is usually present for 2-4 weeks but can last up to 4 months. It may be mildly pruritic. Physical
Causes
DIFFERENTIALSSection 4 of 11
Atopic Dermatitis Keratosis Pilaris
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| Drug Name | Triamcinolone 0.1% cream (Aristocort) |
|---|---|
| Description | Antipruritic effect of this cream is fairly marginal but may be somewhat effective. Treats inflammatory dermatosis that is responsive to steroids. Decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reversing capillary permeability. |
| Adult Dose | Apply a thin film to affected areas bid prn |
| Pediatric Dose | Apply as in adults |
| Contraindications | Documented hypersensitivity; fungal, viral, and bacterial skin infections |
| Interactions | None reported |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Excessive use of topical steroids may cause skin atrophy; do not use in decreased skin circulation; potential for systemic absorption and adrenal suppression when used over large surface areas in young children |
These agents may provide some relief when pruritus is present. They are used to treat minor allergic reactions and anaphylaxis and may be used to pretreat patients with prior documentation of minor allergic reactions. These agents may control itching by blocking effects of endogenously released histamine.
| Drug Name | Hydroxyzine (Atarax, Vistaril) |
|---|---|
| Description | Offers a mild degree of relief from pruritus. Antagonizes H1 receptors in periphery. May suppress histamine activity in the subcortical region of CNS. |
| Adult Dose | 25-50 mg PO q6h prn |
| Pediatric Dose | 1 mg/kg/dose PO q6h prn |
| Contraindications | Documented hypersensitivity; early pregnancy |
| Interactions | May potentiate the effect of opioid analgesics, barbiturates, alcohol, or other CNS depressants |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Associated with clinical exacerbations of porphyria (may not be safe for patients with porphyria); ECG abnormalities (alterations in T waves) may occur; causes drowsiness |
| Media file 1: Multiple erythematous flat-topped papules on the cheeks of an 18-month-old boy with Gianotti-Crosti syndrome. | |
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| Media file 2: Arm of a 3-year-old boy with Gianotti-Crosti syndrome demonstrating well-defined erythematous lichenoid papules on the arm and forearm. | |
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| Media file 3: Gianotti-Crosti syndrome. Thigh of the patient in Image 2. The trunk was strikingly spared. | |
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Gianotti-Crosti Syndrome excerpt
Article Last Updated: Dec 7, 2006
