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
Xanthogranulomas(XG) are benign proliferative disorder of histiocytes, a non-Langerhans cell histiocytosis. Whose etiology is unknown. The nature of these lesions is controversial and could be either reactive or neopl...Xanthogranulomas(XG) are benign proliferative disorder of histiocytes, a non-Langerhans cell histiocytosis. Whose etiology is unknown. The nature of these lesions is controversial and could be either reactive or neoplastic;the presence of monoclonal cells does, however, favor the second hypothesis. Xanthogranuloma is frequently found in young adults and children(under 20 years old), mainly in the skin. In about 5%-10% of all Juvenile XG(JXG) cases xanthogranuloma are extracutaneous. Within this group, the site most frequently involved is the eye. Other involved organs are heart, liver, adrenals, oropharynx, lung, spleen, central nervous system and subcutaneous tissue, although involvement of the spine is uncommon. Isolated lesions involving the sacral region are extremely rare. To date, this is the first reported case of a giant JXG arising from S1 with extension into the pelvic region in an adult spine.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
Introduction Xanthogranuloma is a kind of rare,non-Langerhans cell histiocytosis that most commonly occurs in infancy or early childhood.Adult xanthogranuloma was first described by Gartmann and Tritsch in 1963,and th...Introduction Xanthogranuloma is a kind of rare,non-Langerhans cell histiocytosis that most commonly occurs in infancy or early childhood.Adult xanthogranuloma was first described by Gartmann and Tritsch in 1963,and there have been only approximately one hundred cases reported until now[1].Diagnosis of this disease might be complicated due to its unusual forms,and a group of dermatoses need to be differentiated.We here described a case of multiple adult xanthogranuloma (MAXG) in a young man.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Xanthogranulomas(XG) are benign proliferative disorder of histiocytes, a non-Langerhans cell histiocytosis. Whose etiology is unknown. The nature of these lesions is controversial and could be either reactive or neoplastic;the presence of monoclonal cells does, however, favor the second hypothesis. Xanthogranuloma is frequently found in young adults and children(under 20 years old), mainly in the skin. In about 5%-10% of all Juvenile XG(JXG) cases xanthogranuloma are extracutaneous. Within this group, the site most frequently involved is the eye. Other involved organs are heart, liver, adrenals, oropharynx, lung, spleen, central nervous system and subcutaneous tissue, although involvement of the spine is uncommon. Isolated lesions involving the sacral region are extremely rare. To date, this is the first reported case of a giant JXG arising from S1 with extension into the pelvic region in an adult spine.
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.
文摘Introduction Xanthogranuloma is a kind of rare,non-Langerhans cell histiocytosis that most commonly occurs in infancy or early childhood.Adult xanthogranuloma was first described by Gartmann and Tritsch in 1963,and there have been only approximately one hundred cases reported until now[1].Diagnosis of this disease might be complicated due to its unusual forms,and a group of dermatoses need to be differentiated.We here described a case of multiple adult xanthogranuloma (MAXG) in a young man.
