Pediatrics

Gianotti-Crosti syndrome (papular acrodermatitis)

OVERVIEW: What every practitioner needs to know

Are you sure your patient has Gianotti-Crosti syndrome? What are the typical findings for this disease?

Gianotti-Crosti syndrome is characterized by asymptomatic, discrete, monomorphous, 1-5–mm pink to erythematous papules, favoring the distal extremities and face (Figure 1).

Figure 1.

Typical distribution of Gianotti-Crosti syndrome.

Lesions may develop a more intense erythematous, uriticarial, vesicular or, rarely, hemorrhagic appearance (Figure 2).

Figure 2.

Hemorrhagic Gianotti-Crosti syndrome.

They develop abruptly, last for several weeks, and resolve spontaneously without sequelae.

The trunk is almost always spared (hence the term acrodermatitis) (Figure 3). If there is significant truncal involvement of the cutaneous eruption, the diagnosis should be questioned.

Figure 3.

Truncal sparing in Gianotti-Crosti syndrome.

Patients are usually otherwise well when they present with the characteristic cutaneous findings, although some patients may show signs of associated viral infection, including fever, pharyngitis, hepatomegaly, splenomegaly, lymphadenopathy, or signs of upper respiratory infection.

What other disease/condition shares some of these symptoms?

The clinical differential diagnosis includes:

Henoch-Schönlein purpura (IgA vasculitis)

Septicemia

Irritant or allergic contact dermatitis

Papular eczema

Acute arthropod assault or papular urticaria

Scabies

Molluscum contagiosum (Figure 4)

Figure 4.

Gianotti-Crosti syndrome mimicking molluscum contagiosum.

Histiocytic disease (Langerhans cell histiocytosis, generalized eruptive histiocytosis)

Lichen planus or lichenoid drug eruption

Photosenstitive eruption (photoallergic disease, photocontact disease, polymorphous light eruption)

What caused this disease to develop at this time?

The exact cause of Gianotti-Crosti syndrome is unknown. Most authors consider it to be a viral exanthem, in which virus disseminates to the skin by hematogenous spread, and deposition of circulating immune complexes in epidermis and blood vessels results in the cutaneous findings.

Patients may present with signs of acute viral infection or may give a history of a preceding illness (up to 6 weeks previously). A known history of current or preceding infection may not be present.

What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results?

Laboratory studies generally are not necessary when evaluating a patient with Gianotti-Crosti syndrome.

Hepatitis rarely occurs from the underlying viral illness (specifically in cases of hepatitis B, cytomegalovirus, or EBV). Liver enzyme levels will be elevated in these cases. Patients at high risk for hepatitis B infection should be evaluated with hepatitis serologic tests.

Other common laboratory abnormalities reflect the associated viral infection (lymphocytosis or thrombocytosis).

If there is clinical concern for a specific infectious agent, and reason to document or treat this underlying infection, appropriate serum titers or polymerase chain reaction for these agents can be performed.

Skin biopsy may be performed but is nonspecific. The most common findings are epidermal spongiosis and parakeratosis, with an associated superficial perivascular and interstitial lymphocytic infiltrate. Deep inflammation may also be present. There is often papillary dermal edema, which may be marked. Lymphocytic vasculitis has been reported.

Would imaging studies be helpful? If so, which ones?

Imaging studies are not indicated.

If you are able to confirm that the patient has this disease, what treatment should be initiated?

The cutaneous eruption is self-limited and does not require treatment.

Parents and patients should be reassured that the cutaneous eruption itself is not contagious. It can last several weeks (6-8 weeks in most cases) but may take longer for complete resolution.

In rare cases in which patients complain of significant pruritus, oral antihistamines and soothing topical preparations can be helpful. Topical steroids are generally not helpful or recommended.

Symptoms of the associated infection should be treated as needed (antipyretic drugs, analgesic agents, or appropriate antibiotics as necessary).

What are the possible outcomes of Gianottii-Crosti syndrome?

The long-term prognosis is excellent. Recurrences are extremely rare.

What causes this disease and how frequent is it?

Most authors postulate a postinfectious cause for Gianotti-Crosti syndrome in which dermal deposition of circulating immune complexes results in the cutaneous findings. The cause of the acral distribution has not been fully elucidated.

The most common infectious agent implicated historically is hepatitis B (the agent first described by Gianotti, and the classically termed "papular acrodermatitis of childhood"). Currently, Epstein-Barr virus is the most common predisposing infection documented in the United States. Many other viral and bacterial agents have been implicated, including parvovirus B19, cytomegalovirus, enteroviruses, respiratory viruses, ricketsial infections, streptococcus, and HIV. The cutaneous eruption has also developed after vaccine administration for hepatitis A and B, polio, and influenza, among others.

The rash occurs most commonly in young children (average age of 2 years) but can be seen in adolescents.

Boys and girls are affected equally.

There is no familial or genetic predisposition the development of rash.

There is a seasonal peak in the spring and fall. A preceding or current viral or bacterial illness may be elicited.

How do these pathogens/genes/exposures cause the disease?

Virus affects the skin through hematogenous spread, with epidermal and dermal deposition of immune complexes resulting in the cutaneous findings. The cause of the acral distribution has not been elucidated but may reflect the involvement of smaller, more superficial capillaries in these distal locations.

What complications might you expect from the disease or treatment of the disease?

Complications and long-term sequelae are extremely rare and are associated with the underlying viral illness. Chronic hepatitis from hepatitis B infection can occur.

How can Gianotti-Crosti syndrome be prevented?

Vaccination against hepatitis B and other viruses can prevent the predisposing illness, as can techniques to minimize spread of common respiratory and gastrointestinal viruses (such as good handwashing habits and minimizing exposure to ill individuals).

What is the evidence?

Chuh, AAT. "Diagnostic criteria for Gianotti-Crosti syndrome: a prospective case-control study for validity assessment". Cutis. vol. 68. 2001. pp. 207-13.

Hofmann, R, Schuppe, HC, Adams, O. "Gianotti-Crosti syndrome associated with Epstein-Barr virus infection". Pediatr Dermatol. vol. 14. 1997. pp. 2733-77.

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