Acrodermatitis

Other names

Gianotti-Crosti syndrome

Clinical features

Gianotti Crosti syndrome (GCS) is a rare childhood skin condition characterized by a papular rash with blisters on the skin of the legs, buttocks, and arms.

Age of onset

It typically affects children between 9 months and 9 years of age.

Signs and symptoms

• Skin lesions typically last at least 10 days and often last for several weeks.
• The lesions are usually preceded by an underlying infection (usually a virus), which may cause associated symptoms such as low-grade fever, sore throat, or symptoms of an upper respiratory infection.
• When GCS is associated with hepatitis B, Epstein-Barr, or cytomegalovirus (CMV) infection, acute hepatitis may also occur.
• GCS is thought to be a hypersensitive response to the underlying infection.

Cause

• While in many countries the underlying cause is hepatitis B, this is rarely the cause in North America.
• In Italian children, Gianotti-Crosti syndrome is seen frequently with hepatitis B. But this link is rarely seen in the United States.
• Epstein-Barr virus (EBV, mononucleosis) is the virus most often associated with acrodermatitis.

Pathophysiology

• Gianotti Crosti syndrome (GCS) is thought to be due to a hypersensitive response to a previous infection.
• The underlying infection tends to correlate with the infectious agent endemic to a specific geographic region.
• For example, in Japan and Mediterranean countries, GCS is more commonly associated with hepatitis B virus infection.
• Since there is more universal use of hepatitis B immunization, Epstein-Barr virus is now the most common associated infection worldwide.
• GCS may also occur after coxsackievirus, infectious mononucleosis, cytomegalovirus, enterovirus infections, ECHO viruses, respiratory syncytial virus, or after vaccination with a live virus serum.
• In most cases, no laboratory tests are needed when a person is diagnosed with GCS.

Treatment

• GCS typically does not require treatment and goes away on its own within 1 to 3 months.
• In some cases, a mild topical steroid cream may be prescribed to relieve itching.
• Infections linked with this condition, such as hepatitis B and Epstein-Barr, are treated.
• Cortisone creams and oral antihistamines may help with itching and irritation.

Prognosis

The rash usually disappears on its own in about 3 to 8 weeks without treatment or complication. Associated conditions must be watched carefully.

Complications

Complications occur as a result of associated conditions, rather than as a result of the rash.

Other names

• Papular acrodermatitis of childhood;
• Infantile acrodermatitis;
• Acrodermatitis - infantile lichenoid;
• Acrodermatitis - papular infantile;
• Papulovesicular acro-located syndrome