Juvenile xanthogranuloma (XGJ) is the most common form of non-Langerhansian histiocytosis. We report a pediatric case of multiple XGJ without visceral involvement in a 6-month-old female infant who was hospitalized wi...Juvenile xanthogranuloma (XGJ) is the most common form of non-Langerhansian histiocytosis. We report a pediatric case of multiple XGJ without visceral involvement in a 6-month-old female infant who was hospitalized with disseminated congenital skin nodules, of firm consistency, of variable diameter ranging from a few millimetres to 1 cm, involving the face, scalp, trunk and limbs. There was no mucosal localization. The remainder of the somatic examination was unremarkable. The diagnosis of disseminated XGJ was confirmed by skin biopsy without visceral or systemic involvement. Therapeutic abstention was recommended. This observation underlines the fact that XGJ is a rare clinical entity that should not be overlooked when faced with congenital skin nodules.展开更多
Introduction:Histiocytoses are localized or systemic diseases that can broadly be classified into Langerhans cell histiocytosis and non-Langerhans cell histiocytosis.Juvenile Xanthogranuloma(JXG)belongs to the latter ...Introduction:Histiocytoses are localized or systemic diseases that can broadly be classified into Langerhans cell histiocytosis and non-Langerhans cell histiocytosis.Juvenile Xanthogranuloma(JXG)belongs to the latter group,which occurs in around 0.5%population younger than 5 years,and presents as solitary or multiple lesions.Here,we present a case of disseminated JXG in an infant with the lesions regressing spontaneously.Case presentation:A 7-month-old boy presented with multiple erythematous nodular lesions over the body excluding limbs for 3 months.Initial laboratory work up was normal.Skin histopathology showed a dermal nodule with sheets of histiocytes exhibiting grooved vesicular nuclei and pale eosinophilic cytoplasm along with touton giant cells.Immunohistochemistry markers further confirmed the diagnosis.Lesions healed completely with symptomatic relief in 1.5 years and no recurrence occurred.Discussion:Disseminated JXG is a benign childhood disorder that can sometimes be associated with internal organs involvement mainly bones,eyes,and brain.Serious complications may follow in case of extracutaneous spread.Most frequently,JXG follows the occurrence of another neoplastic disorder but can sometimes appear de novo.Skin biopsy is required for the diagnosis and better education of the family.Conclusion:JXG in infancy is a rarity in South Asia.Therefore,along with the clinical judgment,clinicians must also get histological confirmation in order to manage this disorder successfully.Close surveillance for multiorgan involvement is also required to avoid any irreversible sequalae.展开更多
文摘Juvenile xanthogranuloma (XGJ) is the most common form of non-Langerhansian histiocytosis. We report a pediatric case of multiple XGJ without visceral involvement in a 6-month-old female infant who was hospitalized with disseminated congenital skin nodules, of firm consistency, of variable diameter ranging from a few millimetres to 1 cm, involving the face, scalp, trunk and limbs. There was no mucosal localization. The remainder of the somatic examination was unremarkable. The diagnosis of disseminated XGJ was confirmed by skin biopsy without visceral or systemic involvement. Therapeutic abstention was recommended. This observation underlines the fact that XGJ is a rare clinical entity that should not be overlooked when faced with congenital skin nodules.
文摘Introduction:Histiocytoses are localized or systemic diseases that can broadly be classified into Langerhans cell histiocytosis and non-Langerhans cell histiocytosis.Juvenile Xanthogranuloma(JXG)belongs to the latter group,which occurs in around 0.5%population younger than 5 years,and presents as solitary or multiple lesions.Here,we present a case of disseminated JXG in an infant with the lesions regressing spontaneously.Case presentation:A 7-month-old boy presented with multiple erythematous nodular lesions over the body excluding limbs for 3 months.Initial laboratory work up was normal.Skin histopathology showed a dermal nodule with sheets of histiocytes exhibiting grooved vesicular nuclei and pale eosinophilic cytoplasm along with touton giant cells.Immunohistochemistry markers further confirmed the diagnosis.Lesions healed completely with symptomatic relief in 1.5 years and no recurrence occurred.Discussion:Disseminated JXG is a benign childhood disorder that can sometimes be associated with internal organs involvement mainly bones,eyes,and brain.Serious complications may follow in case of extracutaneous spread.Most frequently,JXG follows the occurrence of another neoplastic disorder but can sometimes appear de novo.Skin biopsy is required for the diagnosis and better education of the family.Conclusion:JXG in infancy is a rarity in South Asia.Therefore,along with the clinical judgment,clinicians must also get histological confirmation in order to manage this disorder successfully.Close surveillance for multiorgan involvement is also required to avoid any irreversible sequalae.
