Gianotti-Crosti Syndrome

REPORT OF A CASE

A 22-month old boy was brought to the University Pediatric Dermatology clinic by his parents for evaluation of a progressive erythematous eruption of 3 weeks duration. The rash was asymptomatic and the child was otherwise well with no history of a preceding fever or upper respiratory tract infection. No other family members were affected. The eruption consisted of erythematous 1-4mm papules over his face, trunk and extremities. The midline area of his chest and abdomen showed only minimal involvement, while the lateral aspects of his trunk were markedly involved and showed coalescence of the papules into raised geographic plaques. Cervical lymphadenopathy was present.
Laboratory evaluation revealed a slight elevation of serum LDH and alkaline phosphatase. The total white blood cell count was normal with a slight lymphocytosis.
A biopsy was taken from an involved area on his trunk.Laboratory evaluation revealed a slight elevation of serum LDH and alkaline phosphatase. The total white blood cell count was normal with a slight lymphocytosis.

A biopsy was taken from an involved area on his trunk.

CLINICAL COURSE: Serologic evaluation was performed for a variety of viruses and revealed a high titer of antibodies to certain components of the Epstein-Barr virus panel consistent with recent infection. On follow up examination one week later his eruption had almost completely cleared.

DISCUSSION: Gianotti-Crosti syndrome is a reactive exanthem that can appear following infection by a number of different viruses. It was first described in association with Hepatitis B infection by Gianotti in 1955.1 Since then, it has been associated with many viruses including Epstein-Barr virus,2 coxsackie virus,3 parainfluenza virus4 and cytomegalovirus,3 among others. It is most commonly seen in children aged 2-6 years and is characterized by the predominantly acral distribution of non-pruritic erythematous papules or papulovesicles in an otherwise healthy child. Frequently, parents will relate the occurrence of upper respiratory tract symptoms preceding the onset of the rash. Lymphadenopathy is often present and hepatomegaly, although less common, can also be seen. The exanthem typically resolves in 3-5 weeks without treatment. Our patient’s clinical presentation was somewhat unusual; his eruption was most extensive over the trunk, an area that is typically spared or shows only minimal involvement. Laboratory evaluation often reveals a mild lymphocytosis (54-73%) and transient elevations of liver enzymes can be present even in the absence of Hepatitis B infection. 4 The histopathologic changes include a superficial or superficial and deep lymphocytic perivascular infiltrate. In older lesions the infiltrate may become interstitial. Spongiosis is uniformly present and may become so extensive that spongiotic vesicles are formed. Edema of the papillary dermis is often profound.

Because of the association with known viruses, it is essential to perform appropriate serologic tests. Hepatitis B, in particular may be present without any other signs or symptoms of infection. Serologic testing in our patient revealed a recent infection with EBV.

 

 

REFERENCES

1 Gianotti F. Rilievi di una particolare casistica tossinfettiva caratterizzata de eruzione eritemato-infiltrativa desquamativa a focolai lenticolari, a sede elettiva acroesposta. G Ital Dermatol 1955;96:678-97.

2 Lowe L, Hebert AA, Duvic M. Gianotti-Crosti syndrome associated with Epstein-Barr virus infection. J Am Acad Dermatol 1989; 20:336-8.

3 Taieb A, Plantin P, du Pasquier P, et al. Gianotti-Crosti syndrome: a study of 26 cases. Br J Dermatol 1986;115:49-59.

4 Spear KL, Winkelmann RK. Gianotti-Crosti Syndrome. Arch Dermatol 1984:1120:891-896.

 

source : http://dermatology.cdlib.org/

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Clinical and Editor in Chief :

WIDODO JUDARWANTO

email : judarwanto@gmail.com,

 

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