Abstract
ABSTRACTObjective: To report a rate case of Juvenile xanthogranuloma in a newborn infant.Case description: We present the case of a 31-week preterm newborn with multiple skin lesions whose clinical, histological and immunohistochemical findings allowed the diagnosis of juvenile xanthogranuloma. Currently, the patient has nine months-old, and there is no aggravation of the skin lesions or evidence of extra-cutaneous involvement, particularly ophthalmic.Comments: Juvenile xanthogranuloma is a rare and benign condition, included in the vast group of non-Langerhans histiocytosis. It typically occurs in the pediatric age and may have a neonatal presentation. It affects predominantly the skin, in the form of papules or yellow and/or erythematous nodules and could be asymptomatic, multiple or solitary. Extra-cutaneous involvement, is more common in toddlers and when multiple lesions are present. The eye is the most affected site. We highlight this clinical case by its presentation in the neonatal period and in the form of multiple lesions, which bestows an increased risk of extra-cutaneous involvement, although this has not yet been verified.
Highlights
Juvenile xanthogranuloma (JXG) is a rare and benign proliferative disease, belonging to the vast group of non-Langerhans histiocytoses (NLH).[1,2,3,4,5,6,7] First described by Adamson in 1905,8 its current nomenclature was adopted in 1954.9With unknown etiology and incidence, it affects predominantly children in the first two years of life, and there has been cases of diagnosis in adulthood.[1,2,3,4,5,6,7] The usual presentation is cutaneous, with extracutaneous involvement being rare.[1,2,3,4,5,6,7,10,11] Diagnostic suspicion is clinically based and corroborated by histology and immunohistochemistry.[2,3,4,5,6]The purpose of this case report is to describe a rare case of juvenile xanthogranuloma in the neonatal period
Diagnostic suspicion is clinically based and corroborated by histology and immunohistochemistry.[2,3,4,5,6]. The purpose of this case report is to describe a rare case of juvenile xanthogranuloma in the neonatal period
The form of multiple lesions occurs in 7 to 10% of the cases, predominating in infants aged less than 6 months, and in males.[3,5,7]
Summary
Juvenile xanthogranuloma (JXG) is a rare and benign proliferative disease, belonging to the vast group of non-Langerhans histiocytoses (NLH).[1,2,3,4,5,6,7] First described by Adamson in 1905,8 its current nomenclature was adopted in 1954.9With unknown etiology and incidence, it affects predominantly children in the first two years of life, and there has been cases of diagnosis in adulthood.[1,2,3,4,5,6,7] The usual presentation is cutaneous, with extracutaneous involvement being rare.[1,2,3,4,5,6,7,10,11] Diagnostic suspicion is clinically based and corroborated by histology and immunohistochemistry.[2,3,4,5,6]The purpose of this case report is to describe a rare case of juvenile xanthogranuloma in the neonatal period. With unknown etiology and incidence, it affects predominantly children in the first two years of life, and there has been cases of diagnosis in adulthood.[1,2,3,4,5,6,7] The usual presentation is cutaneous, with extracutaneous involvement being rare.[1,2,3,4,5,6,7,10,11] Diagnostic suspicion is clinically based and corroborated by histology and immunohistochemistry.[2,3,4,5,6]
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