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Actas Dermo-sifiliograficas Nov 2020Juvenile xanthogranulomas (JXGs) are rare, benign lesions that belong to the large group of non-Langerhans cell histiocytoses. JXG presents with 1 or more erythematous... (Review)
Review
Juvenile xanthogranulomas (JXGs) are rare, benign lesions that belong to the large group of non-Langerhans cell histiocytoses. JXG presents with 1 or more erythematous or yellowish nodules that are usually located on the head or neck. Most JXG lesions are congenital or appear during the first year of life. Extracutaneous involvement is rare, but the literature traditionally suggests investigating the possibility of ocular compromise. JXG is mainly a clinical diagnosis, but a skin biopsy may sometimes be needed for confirmation. JXGs on the skin are self-limiting and usually do not require treatment. This review describes the clinical and therapeutic aspects of JXG, emphasizing available evidence and the diagnosis of extracutaneous involvement.
Topics: Biopsy; Histiocytosis, Non-Langerhans-Cell; Humans; Skin; Xanthogranuloma, Juvenile
PubMed: 32721389
DOI: 10.1016/j.ad.2020.07.004 -
Journal of the American Academy of... Mar 1997Juvenile xanthogranuloma (JXG) is a benign, self-healing disorder characterized by solitary or multiple yellow-red nodules on the skin and, occasionally, in other... (Review)
Review
Juvenile xanthogranuloma (JXG) is a benign, self-healing disorder characterized by solitary or multiple yellow-red nodules on the skin and, occasionally, in other organs. It is predominantly a disease of infancy or early childhood, although adults may also be affected. Histologically, JXG represents an accumulation of histiocytes lacking Birbeck granules (non-Langerhans cells), which can be differentiated from Langerhans cells by specific staining techniques. Affected persons have normal lipid metabolism. JXG is therefore classified as a normolipemic non-Langerhans cell histiocytosis. The patient's general health is not impaired and, in the absence of associated conditions, the prognosis is excellent. Diagnosis is readily made in typical cases, but may be more difficult in unusual variants.
Topics: Adult; Child; Diagnosis, Differential; Humans; Prognosis; Xanthogranuloma, Juvenile
PubMed: 9091465
DOI: 10.1016/s0190-9622(97)80207-1 -
Pediatric Dermatology Jan 2022
Topics: Humans; Xanthogranuloma, Juvenile
PubMed: 35106819
DOI: 10.1111/pde.14920 -
La Clinica Terapeutica 2022Neurofibromatosis type 1 (NF1), is a rare genetic disorder that may involve almost every organ system in the body such as cutaneous, ophthalmologic and central and... (Review)
Review
Juvenile xanthogranuloma in neurofibromatosis type 1. Prevalence and possible correlation with lymphoproliferative diseases: experience of a single center and review of the literature.
Neurofibromatosis type 1 (NF1), is a rare genetic disorder that may involve almost every organ system in the body such as cutaneous, ophthalmologic and central and peripheral nervous system. Cutaneous findings are usually the first sign of the disease. In this study, we investigate the real prevalence of xanthogranulomas juvenile (JXG) and possible correlation with lymphoproliferative diseases. This is a retrospective study conducted on a population with NF1 followed by February 1983 to February 2022 at the "Sapienza" University of Rome, Italy. We investigate the real prevalence of juvenile xanthogranuloma in NF1 and possible correlation with lymphoproliferative diseases. JXG was present in 39 cases (3.1%). JXG is more frequent in NF1 than in the general population while the possible association with lymphoproliferative diseases in NF1 remains controversial.
Topics: Humans; Neurofibromatosis 1; Prevalence; Retrospective Studies; Skin; Xanthogranuloma, Juvenile
PubMed: 35857053
DOI: 10.7417/CT.2022.2445 -
Cutis Oct 1994
Review
Topics: Diagnosis, Differential; Humans; Prognosis; Xanthogranuloma, Juvenile
PubMed: 7805405
DOI: No ID Found -
Dermatology Online Journal Mar 2024Juvenile xanthogranuloma is the most frequent form of non-Langerhans cell histiocytosis in children. Clinically, it presents as well defined, yellowish papules that are...
Juvenile xanthogranuloma is the most frequent form of non-Langerhans cell histiocytosis in children. Clinically, it presents as well defined, yellowish papules that are typically located on the head, neck, upper trunk, and proximal region of the extremities. Although solitary lesions are the most common presentation, few cases of multiple juvenile xanthogranuloma have been described, more frequently associated with extracutaneous involvement. We report a 2-month-old girl with 22 cutaneous papules, clinically and histologically compatible with juvenile xanthogranulomas. Screening of visceral involvement was performed with no evidence of systemic disease. Identifying high-risk factors of systemic disease in patients with multiple juvenile xanthogranuloma is essential to perform an appropriate management of this entity.
Topics: Humans; Xanthogranuloma, Juvenile; Female; Infant
PubMed: 38762861
DOI: 10.5070/D330163291 -
Pediatric Radiology Feb 2023Juvenile xanthogranuloma is rare in children and there are limited data on its imaging features.
BACKGROUND
Juvenile xanthogranuloma is rare in children and there are limited data on its imaging features.
OBJECTIVE
To analyze the computed tomography (CT) and magnetic resonance imaging (MRI) features of juvenile xanthogranuloma in children.
MATERIALS AND METHODS
A retrospective review was performed of clinical and radiographic data of histologically confirmed juvenile xanthogranuloma between January 2009 and June 2020.
RESULTS
Fourteen children (4 girls, 10 boys; age range: 1 day to 13 years, mean age: 73 months) were included in the study: 4/14 had CT only, 5/14 had MRI only and 5/14 had CT and MRI. Sites of extracutaneous juvenile xanthogranuloma involvement included subcutaneous soft tissue (8/14), liver (2/14), lungs (2/14), kidney (2/14), nose (2/14), pancreas (1/14), central nervous system (1/14) and greater omentum (1/14), mainly manifested as single or multiple nodules or masses in different organs. On CT, the lesions mainly manifested as an iso-hypo density mass with mild or marked enhancement. On MRI, the lesions mainly manifested as slightly hyperintense on T1 and slightly hypointense on T2, with decreased diffusivity and homogeneous enhancement. Juvenile xanthogranuloma was not included in the imaging differential diagnosis in any case.
CONCLUSION
Juvenile xanthogranuloma mainly manifests as single or multiple nodules or masses in different organs. Slight hyperintensity on T1 and slight hypointensity on T2 with decreased diffusivity and homogeneous enhancement are relatively characteristic imaging findings of juvenile xanthogranuloma. Combined with its typical skin lesions and imaging features, radiologists should include juvenile xanthogranuloma in the differential diagnosis when confronted with similar cases.
Topics: Male; Child; Female; Humans; Infant; Tomography, X-Ray Computed; Xanthogranuloma, Juvenile; Magnetic Resonance Imaging; Retrospective Studies; Diagnosis, Differential
PubMed: 36040525
DOI: 10.1007/s00247-022-05486-5 -
Asian Journal of Surgery Jun 2023
Topics: Humans; Xanthogranuloma, Juvenile; Magnetic Resonance Imaging
PubMed: 36473813
DOI: 10.1016/j.asjsur.2022.11.121 -
Indian Pediatrics Jun 2022
Topics: Glottis; Humans; Xanthogranuloma, Juvenile
PubMed: 35695144
DOI: No ID Found -
Indian Pediatrics Nov 2018
Topics: Child, Preschool; Diagnosis, Differential; Exanthema; Humans; Male; Skin; Xanthogranuloma, Juvenile
PubMed: 30587663
DOI: No ID Found