GIANOTTI-CROSTI SYNDROME

 

Epidemiology

 

GCS, also known as infantile papular acrodermatitis and papular acrodermatitis of childhood, is a common, self-limited dermatosis seen worldwide. It affects infants and children between the ages of 6 months and 12 years with the peak age of presentation being 1 to 6 years of age. In children, there is neither an ethnic nor sexual predilection. There have been only scattered case reports in adults.

 

Etiology and Pathogenesis

 

GCS is a cutaneous reaction pattern associated with viruses, bacteria, and vaccines. Historically, it was thought to be induced exclusively by hepatitis B, but over time other infectious triggers were identified. When hepatitis B is the causative agent, sub-type ayw is most frequently seen. Associated triggers include hepatitis B and EBV most commonly, but other associated viruses include cytomegalovirus, enterovirus, respiratory syncytial virus, rotavirus, adenovirus, echovirus, pox virus, poliovirus, coxsackie virus, parvovirus, HIV, hepatitis A, hepatitis C, and parainfluenza virus. Bacterial pathogens include Mycoplasma pneumoniae, Borrelia burgdorferi, Bartonella henselae, and group A β-hemolytic streptococcus. Immunizations that have been associated include influenza, diphtheria, tetanus, pertussis, bacillus Calmette-Guérin, Haemophilus influenzae type b, and oral polio vaccine. The exact pathogenesis of GCS is not known but may be due to a viral antigenemia or circulating immune complexes. Virus-like particles have been seen in cytoplasmic vacuoles and lysosomes in lesional skin.

 

GIANOTTI-CROSTI SYNDROME AT

AGLANCE

• Papular acrodermatitis of childhood.
• Common, self-limited dermatosis.
• Monomorphic dome-shaped or flat-topped papules symmetrically distributed on face and extensor extremities.
• Associated with multiple viral triggers and immunizations.
• Historically associated with hepatitis B infection, but now more often triggered by Epstein-Barr virus.

 

Clinical Findings

 

HISTORY

 

Before the onset of the exanthem, a non-specific prodrome of upper respiratory tract infection with fever and pharyngitis plus low-grade fever may be present.

 

CUTANEOUS LESIONS

 

Typically, patients develop multiple coalescing, monomorphous, flat-topped or dome-shaped, red-brown papules and papulovesicles . Lesions can be pruritic and, rarely, hemorrhagic. Papules vary from 1 to 10 mm in diameter  and are distributed symmetrically on the cheeks, extensor surfaces of the extremities, and the buttocks. The trunk, palms, and soles are usually, but not always,

spared. Occasionally, the small papules coalesce into large plaques. Cutaneous lesions evolve over a few days and last for 2 to 8 weeks.

 

RELATED PHYSICAL FINDINGS

 

Constitutional symptoms, including malaise, low-grade fevers, and diarrhea, are sometimes seen at time of presentation, but are usually mild. Patients can develop lymphadenopathy, especially of the cervical, axillary, and inguinal chains.

 

Laboratory Tests

 

In most patients, the diagnosis is established on clinical findings and no further diagnostic testing is necessary. In patients with hepatomegaly, further diagnostic testing, including complete blood cell count and liver enzymes, may be warranted. Lymphocytosis or lymphopenia are common and do not require further diagnostic evaluation. If there are elevations in the liver enzymes, evaluation for hepatitis or EBV should be performed. If hepatitis is suggested, checking anti-hepatitis A IgG and IgM, hepatitis B surface antigen and core antibody, and anti-hepatitis C IgG is indicated.

Differential Diagnosis

 

Complications

 

Complications are rare with the majority of cases having a self-limited and mild course. On rare occasions, when GCS is associated with hepatitis B infection, chronic liver disease with subsequent liver failure can occur.

Prognosis and Clinical Course

In the vast majority of cases, GCS is a self-limited, benign process. The course is quite variable, with the skin findings lasting anywhere from 5 days to 12 months. Lesions heal without scarring, and, rarely, post-inflammatory hypopigmentation or hyperpigmentation is seen. Lymphadenopathy can last for several months. In cases associated with hepatitis B, an anicteric viral hepatitis evolves and may not be seen until 2 weeks after cutaneous lesions are seen. Most of the time, the hepatitis is self-limited.

 

Treatment

 

No treatment is necessary in the majority of cases. In some patients, medium potency topical steroids may decrease the duration of lesions when applied once daily for 1 to 2 weeks. However, patients should be monitored closely because worsening of findings with topical steroid use has been documented.¹³⁶ In severe cases, systemic, pulsed corticosteroids may be administered. Oral antihistamines or topical anti-itch lotions may diminish severe pruritus.

Box 192-8 Differential Diagnosis of Gianotti-Crosti Syndrome

Most Likely

• Papular urticaria
• Pityriasis rosea

Consider

• Erythema multiforme
• Lichen planus
• Lichenoid drug eruption
• Pityriasis lichenoides et varioliformis acuta
• Molluscum contagiosum
• Hand-foot-mouth disease
• Follicular eczema
• Erythema infectiosum

Always Rule Out

• Infectious mononucleosis
• Henoch-Schönlein purpura
• Scabies

Prevention

There is no known way to prevent GCS.