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American Journal of Medical Genetics.... Dec 2021
Topics: Adolescent; Child; Child, Preschool; Female; Genetic Predisposition to Disease; Humans; Infant; Male; Neurofibromatosis 1; Risk Factors; Xanthogranuloma, Juvenile
PubMed: 34250743
DOI: 10.1002/ajmg.a.62420 -
Acta Neurochirurgica Jun 2013
Review
Topics: Humans; Infant; Magnetic Resonance Imaging; Male; Skull Base Neoplasms; Treatment Outcome; Xanthogranuloma, Juvenile
PubMed: 23605373
DOI: 10.1007/s00701-013-1718-9 -
Pediatric Dermatology Mar 1993An 18-year-old man has been followed in our department since age 10 years when he began to develop numerous yellow, papular lesions on the scalp, face, neck, trunk, and... (Review)
Review
An 18-year-old man has been followed in our department since age 10 years when he began to develop numerous yellow, papular lesions on the scalp, face, neck, trunk, and upper extremities. The lesions enlarged slowly to form yellow-brown nodules measuring up to 6 cm. During this period five of the nodules and two small papules were excised. Histopathology was consistent with juvenile xanthogranuloma (JXG). Recently, the patient complained of significant loss of memory, and a computed tomography scan was performed showing several cerebral and cerebellar lesions. The cutaneous lesions in our patient were almost identical to those described elsewhere as being characteristic of progressive nodular histiocytoma. The many clinical and histopathologic similarities between lesions of progressive nodular histiocytoma and JXG suggest that they may represent a continuum rather than two distinct disease processes. Given the fact that there are no histopathologic differences with JXG, as well as the wide range of clinical lesions that JXG may adopt, there is no reason to separate the entities. Although JXG lesions are usually believed to be benign and self-healing, large nodular forms can be associated with visceral lesions. Only four previous cases of cutaneous JXG with central nervous system involvement were found in a review of the literature.
Topics: Brain Diseases; Child; Humans; Male; Xanthogranuloma, Juvenile
PubMed: 8493173
DOI: 10.1111/j.1525-1470.1993.tb00018.x -
Ophthalmology Sep 2019
Topics: Dexamethasone; Eyelid Diseases; Glucocorticoids; Humans; Infant, Newborn; Injections, Intralesional; Male; Xanthogranuloma, Juvenile
PubMed: 31443784
DOI: 10.1016/j.ophtha.2019.05.011 -
International Journal of Dermatology May 2021Juvenile xanthogranuloma (JXG) is a rarely encountered skin disorder, which is characterized by the proliferation of non-Langerhans cell histiocytes. As JXG primarily...
BACKGROUND/OBJECTIVE
Juvenile xanthogranuloma (JXG) is a rarely encountered skin disorder, which is characterized by the proliferation of non-Langerhans cell histiocytes. As JXG primarily affects infants and young children, this study aims to describe the epidemiologic, clinical, and histopathologic characteristics of 44 children diagnosed with JXG at a tertiary health care center.
METHODS
Fourty-four children with a histopathologic diagnosis of JXG between January 2003 and January 2017 were retrospectively reviewed. Data related to epidemiologic, clinical, and histopathologic features were extracted from hospital records.
RESULTS
The mean age of the affected patients was 4.6 years old (range: 0-17 years old) at the time of diagnosis. Twenty-five patients (56.8%) were male, and 19 patients were female (43.2%). Thirty-six children (81.8%) had solitary JXG, one of which was a giant congenital JXG; eight children (18.2%) had eruptive JXG. The heterozygote mutation associated with neurofibromatosis 1 gene was detected in one patient who had both eruptive JXG and numerous café-au-lait spots. Another patient with eruptive JXG was identified to have hypercholesterolemia. None of the children with eruptive JXG developed symptoms or signs of extracutaneous involvement during their clinical follow-up.
CONCLUSION
Since JXG is rarely encountered, there may be a tendency toward over-treatment, given concerns for extracutaneous involvement. However, our review revealed no instances of extracutaneous involvement.
Topics: Adolescent; Child; Child, Preschool; Female; Histiocytes; Humans; Infant; Infant, Newborn; Male; Mutation; Retrospective Studies; Tertiary Care Centers; Xanthogranuloma, Juvenile
PubMed: 33301172
DOI: 10.1111/ijd.15223 -
BMC Pediatrics Apr 2019Juvenile xanthogranuloma (JXG) belongs to the heterogeneous group of non-Langerhans cell histiocytosis and is caused by an accumulation and proliferation of macrophages.... (Review)
Review
BACKGROUND
Juvenile xanthogranuloma (JXG) belongs to the heterogeneous group of non-Langerhans cell histiocytosis and is caused by an accumulation and proliferation of macrophages. In the majority of cases JXG is a disorder of early childhood presenting during the first 2 years of life. The typical presentation is a solitary reddish or yellowish skin papule or nodule with spontaneous regression and no need for treatment.
CASE PRESENTATION
Two infants with an atypical presentation of JXG, one with multiple blueberry muffin rash-like skin lesions and the other with severe multi-systemic involvement, are reported. Diagnosis was established by skin biopsy including histological work-up and immunostaining, where markers for macrophages (CD68 and CD163) exhibited significant reactivity.
CONCLUSION
JXG is the most common of the non-Langerhans cell histiocytosis. The typical presentation is a solitary skin lesion. The purpose of this report is to familiarize paediatricians with an unusual variant of this entity in order to facilitate early diagnosis and raise awareness for possible visceral complications and associated medical conditions.
Topics: Antineoplastic Combined Chemotherapy Protocols; Biopsy, Needle; Female; Follow-Up Studies; Humans; Immunohistochemistry; Infant; Magnetic Resonance Imaging; Male; Risk Assessment; Time Factors; Treatment Outcome; Watchful Waiting; Xanthogranuloma, Juvenile
PubMed: 31018833
DOI: 10.1186/s12887-019-1490-y -
JAMA Ophthalmology Mar 2020
Topics: Administration, Ophthalmic; Adrenal Cortex Hormones; Diagnosis, Differential; Eye Diseases; Humans; Male; Predictive Value of Tests; Treatment Outcome; Xanthogranuloma, Juvenile; Young Adult
PubMed: 31999299
DOI: 10.1001/jamaophthalmol.2019.4982 -
Annales de Dermatologie Et de... Jan 2009
Topics: Child; Diagnosis, Differential; Eye Diseases; Humans; Leukemia, Myelomonocytic, Juvenile; Neurofibromatosis 1; Prognosis; Xanthogranuloma, Juvenile
PubMed: 19171239
DOI: 10.1016/j.annder.2008.06.014 -
The American Journal of Dermatopathology Jun 2021
Topics: Child; Elastic Tissue; Female; Humans; Phagocytosis; Xanthogranuloma, Juvenile
PubMed: 33201014
DOI: 10.1097/DAD.0000000000001848 -
Journal of Stomatology, Oral and... Oct 2022Juvenile xanthogranuloma, a form of non-Langerhans cell histiocytosis can be defined as the proliferation of cells with macrophage like characteristics. It has been...
Juvenile xanthogranuloma, a form of non-Langerhans cell histiocytosis can be defined as the proliferation of cells with macrophage like characteristics. It has been described as a benign, asymptomatic and common self-healing disorder of non-Langerhans cell histiocytosis (LCH), affecting mostly infants, children and rarely adults. We have documented a case of a 40 year old male who presented to us with extensive insidious papulonodulous growth over the face. The facial disfigurement caused was catastrophic. An extensive surgical excision and reconstruction was performed followed by histopathological evaluation. Microscopic study and immune histochemistry revaled Juvenile Xanthogranuloma of adult. The article highlights the presentation, diagnosis and management of this mammoth, rare disease.
Topics: Adult; Humans; Male; Xanthogranuloma, Juvenile
PubMed: 35395418
DOI: 10.1016/j.jormas.2022.03.019