Gianotti-Crosti Syndrome
A 10-month-old infant presented with a 3- to 4-day history of a rash on her arms, legs, and ankles bilaterally. Her mother reported that the girl had a reduced appetite 1 week prior and had a fever for 2 days just before the rash broke out. The girl’s mother also mentioned that the rash had begun with a small red dot, then had become a blister and had spread to the arms and legs. She denied any other symptoms in the girl, and there was no history of travel.
The infant did not seem to be bothered by the rash, and there had been no scratching, no medications given, and no sick contacts. Her vaccinations were up-to-date, and there was no family history of similar lesions.
Physical examination revealed an afebrile, well-appearing, and comfortable infant with multiple erythematous and hemorrhagic maculopapular lesions on her elbows, hands, knees, legs, and ankles bilaterally. The girl received a clinical diagnosis of Gianotti-Crosti syndrome (GCS).
GCS is a rare, self-limited eruption that usually is associated with viral infections. In the 1950s, Gianotti and Crosti first described GCS as an exanthem associated with hepatitis B virus, which they termed papular acrodermatitis of childhood.1-3
Because it is benign and self-limited, most cases of GCS go unreported, making the overall incidence unknown.4 It has been suggested that the incidence of GCS parallels that of precipitating infections in specific geographic regions.4
GCS can affect children between 3 months and 15 years of age but occurs primarily in children between 1 and 6 years old. Boys and girls are affected with equal frequency, but adult cases have been reported almost exclusively among women, suggesting that hormones influence the occurrence of GCS. There is no racial or ethnic predilection.
Because of routine universal hepatitis B vaccination during infancy, Epstein-Barr virus (EBV) infection is a more common cause worldwide; in the United States, EBV is responsible for as many as three-quarters of cases.5-8 Other associated infectious agents include adenovirus, rotavirus, hepatitis A and C, parainfluenza, coxsackievirus, respiratory syncytial virus, cytomegalovirus, parvovirus, mumps virus, human herpesvirus 6, HIV, group A streptococcus, Mycoplasma pneumoniae, and Bartonella. Administration of certain vaccines also has been associated with cases of GCS.
Our patient’s case of GCS eventually was found to have been caused by coxsackievirus serotypes B1 and B3.
GCS often is preceded by an upper respiratory infection. The condition usually begins with mild systemic symptoms such as, malaise, low-grade fever, lymphadenopathy, hepatosplenomegaly, and diarrhea. The lesions of GCS most often occur on the face, buttocks, and extremities. Although the lesions of GCS typically are nonpruritic, there are some reports of pruritus in the later stages of the rash. The only significant morbidity involves the underlying infectious process, particularly hepatitis B virus infection.
GCS is a clinical diagnosis, and no laboratory studies are necessary. The condition requires no treatment other than supportive therapies such as topical agents or systemic antihistamines for relief of pruritus.
Our patient’s rash disappeared without any treatment after a week.
References
1. Gianotti F. Report on a special case of toxic infection characterized by a desquamative erythemato-infiltrative eruption with lenticular foci and a selective localization at the extremities [in Italian]. Soc Ital Dermatol Sifilogr Sezioni Interprov Soc Ital Dermatol Sifilogr. 1955;96(6):678-697.
2. Crosti A, Gianotti F. Dermatosi infantile eruttiva acroesposta di probabile origine virosica. Minerva Dermatol. 1956;31(suppl 12):483-486.
3. Crosti A, Gianotti F. Eruptive dermatosis of probable viral origin situated on the acra [in French]. Dermatologica. 1957;115(5):671-677.
4. Craig-Müller SA. Gianotti-Crosti syndrome. Medscape. http://emedicine.medscape.com/article/911275-overview. Updated August 1, 2014. Accessed August 20, 2015.
5. Taïeb A, Plantin P, Du Pasquier P, Guillet G, Maleville J. Gianotti-Crosti syndrome: a study of 26 cases. Br J Dermatol. 1986;115(1):49-59.
6. Smith KJ, Skelton H. Histopathologic features seen in Gianotti-Crosti syndrome secondary to Epstein-Barr virus. J Am Acad Dermatol. 2000;43(6):1076-1079.
7. Drago F, Crovato F, Rebora A. Gianotti-Crosti syndrome as a presenting sign of EBV-induced acute infectious mononucleosis. Clin Exp Dermatol. 1997;22(6):301-302.
8. Hofmann B, Schuppe HC, Adams O, Lenard HG, Lehmann P, Ruzicka T. Gianotti-Crosti syndrome associated with Epstein-Barr virus infection. Pediatr Dermatol. 1997;14(4):273-277.