Dermatology

Gianotti-Crosti Syndrome (Papular acrodermatitis of childhood)

Are You Confident of the Diagnosis?

Gianotti-Crosti syndrome is a relatively common dermatosis that occurs worldwide. The diagnosis is made clinically.

Characteristic findings on physical examination

Gianotti-Crosti syndrome is characterized by the acute onset of monomorphous, pink to red-brown papules or papulovesicles that may become confluent (Figure 1). The papules are distributed symmetrically on the face (Figure 2), extensor surfaces of the extremities, and buttocks, and have occasionally been described as lentil-shaped. The rash may be slightly pruritic, is typically nonhyperkeratotic, and spares the trunk, palms of the hands, and soles of the feet. It may persist for many weeks.

Figure 1.

Multiple 1-2mm monomorphous pink papules coalescing into plaques.

Figure 2.

1-2mm pink papules on the cheek.

The cutaneous manifestations can be preceded by diarrhea or an upper respiratory tract infection. The rash may be accompanied by a low-grade fever, hepatomegaly, splenomegaly, and inguinual or axillary lymphadenopathy. The rash of Gianotti-Crosti may display the koebnerization phenomenon.

Diagnosis confirmation

There is no definitive serologic testing to confirm the diagnosis. Differential diagnosis includes the conditions below.

Erythema multiforme causes target-shaped papular and vesiculobullous lesions on the extremities or mucous membranes. The lesions are often distributed symmetrically, and may be preceded by fever or upper respiratory tract symptoms. The palms and soles are often involved; initial involvement most commonly occurs on the dorsal hands. The condition typically affects adolescents and young adults, and is usually triggered by recurrent herpes simplex virus (HSV) infections. More serious forms that include mucosal involvement have been associated with various infections and medications.

Lichen planus is an acute or chronic inflammatory dermatosis of unknown origin involving skin or mucous membranes. Although the etiology is unknown, there is an association with hepatitis C virus. The condition is uncommon in children and is characterized by intensely pruritic, flat-topped, pink to violaceous, shiny, polygonal papules; fine, linear overlying white scales (Wickham’s striae) may develop. Lichen planus usually occurs on the wrist, shins, scalp, lumbar region, glans penis, or mucous membranes. It rarely involves the cheeks, and there are no systemic manifestations.

Papular urticaria is characterized by chronic or recurrent eruptions of intensely pruritic papules, vesicles, and wheals caused by a hypersensitivity reaction to insect bites. The lesions are usually grouped in a linear cluster on exposed skin, but spare the genital, perianal, and axillary regions.

Although papular urticaria can occur in all age groups, it predominantly affects children. Intense pruritus and excoriations may be present. The rash of papular urticaria resolves much sooner than that of Gianotti-Crosti syndrome. Papular urticaria is diagnosed in patients of appropriate age with symmetrically distributed lesions, a history of hypersensitivity, and exposure to animals or insects.

Scabies is caused by the Sarcoptes scabiei mite and is spread through skin-to-skin contact. Intense pruritus, the most prevalent symptom, is usually worse at night and spares the face and head. The mite often infests the skin of the finger webs, wrist, elbows, axillae, buttocks, and genitalia. Cutaneous manifestations include inflammatory papules, burrows, nodules, and excoriations.

Who is at Risk for Developing this Disease?

The condition affects children 3 months to 15 years of age, but typically occurs before 4 years of age. However, it has also been reported in adults.

What is the Cause of the Disease?

Etiology

Pathophysiology

Although the exact pathogenesis is unclear, Gianotti-Crosti syndrome is considered a self-limited, cutaneous response to various immunologic triggers. Although the syndrome was first described in association with hepatitis B virus (HBV), Epstein-Barr virus (EBV) is now the most common associated virus in the United States.

Hepatitis A virus, cytomegalovirus (CMV), coxsackievirus, respiratory syncytial virus (RSV), parainfluenza virus, rotavirus, mumps, parvovirus, molluscum contagiosum, and childhood immunizations have also been associated with the syndrome.

Recent reports indicate that bacterial infections such as Bartonella henselae, Mycoplasma pneumoniae, and group A streptococci may trigger Gianotti-Crosti syndrome. Although the specific reason that children develop Gianotti-Crosti syndrome in unknown, an association with atopy has been suggested.

Systemic Implications and Complications

Systemic findings are uncommon in this condition. Patients may represent with malaise, low-grade fever, or diarrhea. Cervical, axillary, or inguinal lymphadenopathy may be occasionally found. Rarely, patients may develop hepatomegaly, hepatitis, or splenomegaly secondary to underlying HBV, EBV, or CMV infections.

Treatment Options

Treatment options are summarized in Table I.

Table I.

Treatment options for Gianotti-Crosti syndrome.
Medical Treatment Surgical Procedures Physical Modalities
Topical mid- potency corticosteroids  None Supportive care
- Systemic corticosteroids - Oral antihistamines -Polidocanol    

Optimal Therapeutic Approach for this Disease

Because the condition is self-limited, treatment usually is not required. However, topical steroids and oral antihistamines can be prescribed as needed for pruritus. The optimal therapeutic approach is as follows:

  • Explain the natural history of the Gianotti-Crosti syndrome to the patient and parents so that they understand that improvement may occur after a prolonged time without any treatment, and that most therapeutic modalities work slowly.

  • Start mild topical steroids such as hydrocortisone 1% ointment or cream, initially twice a day to control pruritis.

  • ncrease to mid-potent topical steroid ointment or cream such as triamcinalone (0.1% or 0.5%) or fluocinonide (0.5%) once a day should pruritus persist.

  • Prescribe oral antihistamines for symptomtaic relief of the itching. Hydroxazine or diphenhydramine, dosed appropriately for children, can be used, although this may be unnecessary.

It is not necessary to check for all the potential causes of Gianotti-Crosti. Specific viruses and bacterial causes can be checked for in the approriate setting. HBV and EBV are the only two that should probably be checked routinely. If a patient is felt to have a reaction from a vaccination, referral to an allergist is helpful.

Patient Management

Explain or parents the benign natural history of Gianotti-Crosti syndrome to the patientand parents before beginning any type of treatment. This is a condition that improves without any treatment. Accordingly, therapies are directed toward symptom relief.

Encourage patients to persist with treatment of symptoms; they will improve over time. Regular follow-up is recommended only if coexisiting hepatitis infection is present (refer to hepatology). Most importantly, reassure the patientand parents that Gianotti-Crosti is not dangerous.

Unusual Clinical Scenarios to Consider in Patient Management

Recently, there have been reports of atypical cases of Gianotti-Crosti syndrome occuring in patients coinfected with both human immunodeficiency virus (HIV) and HBV. Also, this condition has been seen in a patient with HSV 1 gingivostomatitis.

Rarely, cases in adults have been described.

What is the Evidence?

Brandt, O, Abeck, D, Gianotti, R, Burgdorf, W. "Gianotti-Crosti syndrome". J Am Acad Dermatol. vol. 54. 2006. pp. 136-45.

(An outstanding review of the clinical presentations, pathogenesis, and management of all aspects of the Gianotti-Crosti syndrome)

Draelos, ZK, Hansen, RC, James, WD. "Gianotti-Crosti syndrome associated with infections other than hepatitis B". JAMA. vol. 256. 1986. pp. 2386-8.

(Cases of Gianotti-Crosti syndrome are rarely associated with HBV in North America. The author examined nine patients who clinically presented with Gianotti-Crosti, and found the underlying causes to vary among RSV , group A beta-hemolytic streptococci, and EBV.

Ricci, G, Patrizi, A, Neri, I, Specchia, F, Tosti, G, Masi, M. "Gianotti-Crosti syndrome and allergic background". Acta Derm Vernereol. vol. 83. 2003. pp. 202-5.

(This study was conducted to see if there was a correlation between Gianotti-Crosti syndrome and children with allergic background. The authors found the presence of atopic dermatitis was significantly higher in those children with Gianotti-Crosti syndrome . In addition, family history of atopy and an increase in IgE levels were seen more frequently and in higher levels in those with Gianotti-Crosti syndrome .)

Atanasovski, M, Dele-Michael, A, Dasgeb, B, Ganger, L, Mehregan, D. "A case report of Gianotti-Crosti post vaccination with MMR and dTAP". Int J Dermatol. vol. 50. 2011. pp. 609-10.

(A new reported case of children developing Gianotti-Crosti syndrome two days after vaccination with MMR and dTAP)

Cocciolone, R, Morey, A, Panasiuk, P, Whitfeld, MJ. "Atypical Gianotti-Crosti syndrome in two HIV and hepatitis B co-infected adults". Australas J Dermatol. vol. 52. 2011. pp. 32-6.

(This article reports an atypical presentation of Gianotti-Crosti syndrome in two 43-year-old patients who had underlying HBV and HIV infections.)

Dauendorffer, JN, Dupuy, A. "Gianotti-Crosti syndrome associated with herpes simplex virus type 1 gingivostomatitis". J Am Acad Dermatol. vol. 64. 2011. pp. 450-1.

(A unique case of Gianotti-Crosti syndrome associated with HSV-1 gingivostomatitis. The authors contend that HSV-1 may be an under-diagnosed cause for Gianotti-Crosti syndrome in children, as HSV-1 infections are often asymptomatic during childhood.)

Xia, Y, Davies, BE, Gunning, S. "Pruritic acral rash in a child". Am Fam Physician. vol. 78. 2008. pp. 103-5.

(A good overview of the clinical presentation and the differential diagnosis of patients with Gianotti-Crosti syndrome)
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