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Case Reports in Dermatology 2021Juvenile xanthogranuloma (JXG) is the most common type of non-Langerhans cell histiocytosis. JXG is a rare benign tumor, which may be present at birth or develop later....
Juvenile xanthogranuloma (JXG) is the most common type of non-Langerhans cell histiocytosis. JXG is a rare benign tumor, which may be present at birth or develop later. The classical form of JXG is characterized by a red-yellowish benign papule or nodule with predilection sites on the head, neck, and trunk, although lesions can appear on extremities or extracutaneous sites. In most cases there is only one lesion, whereas numerous papules or nodules may occur. Special forms of JXG such as mixed, giant, subcutaneous, eruptive, clustered, and plaque-like have been reported and associations between JXG and systemic diseases have been made. Diagnosis mainly relies on the clinical appearance, and histology usually can confirm the disease. Here we present a very rare case of symmetrical giant facial plaque-type juvenile xanthogranuloma (SGFP-JXG) and compare it with classical JXG, variations of JXG, and discuss the differential diagnosis. A 4-year-old Caucasian female presented with plaque-like lesions composed of yellowish confluent papules on both the cheeks. The histological evaluation revealed a histiocytic lesion with a formation of Touton giant cells and immunohistochemistry results confirmed the diagnosis of the SGFP-JXG. In comparison to classical JXG, the onset of SGFP-JXG sometimes occurs later and the spontaneous resolution period may be prolonged. No associated diseases and no systemic involvements were observed. Histopathology is required to differentiate this form of JXG from other histiocytosis. To the best of our knowledge, only four cases of SGFP-JXG have been reported in the literature so far.
PubMed: 34413740
DOI: 10.1159/000515151 -
Revista Paulista de Pediatria : Orgao... 2019To report a rate case of Juvenile xanthogranuloma in a newborn infant.
OBJECTIVE
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.
Topics: Biopsy; Diagnosis, Differential; Gestational Age; Humans; Immunohistochemistry; Infant; Male; Patient Care; Xanthogranuloma, Juvenile
PubMed: 30810696
DOI: 10.1590/1984-0462/;2019;37;2;00013 -
Advances in Immunology 2013Histiocytic disorders represent a group of complex pathologies characterized by the accumulation of histiocytes, an old term for tissue-resident macrophages and... (Review)
Review
Histiocytic disorders represent a group of complex pathologies characterized by the accumulation of histiocytes, an old term for tissue-resident macrophages and dendritic cells. Langerhans cell histiocytosis is the most frequent of histiocytosis in humans and has been thought to arise from the abnormal accumulation of epidermal dendritic cells called Langerhans cells. In this chapter, we discuss the origin and differentiation of Langerhans cells and dendritic cells and present accumulated evidence that suggests that Langerhans cell histiocytosis does not result from abnormal Langerhans cell homeostasis but rather is a consequence of misguided differentiation programs of myeloid dendritic cell precursors. We propose reclassification of Langerhans cell histiocytosis, juvenile xanthogranuloma, and Erdheim-Chester disease as inflammatory myeloid neoplasias.
Topics: Animals; Cell Differentiation; Dendritic Cells; Histiocytosis; Humans
PubMed: 24070383
DOI: 10.1016/B978-0-12-417028-5.00005-3 -
Canadian Association of Radiologists... May 2016Pulmonary opacities/nodules are common findings on computed tomography examinations, which may represent an underlying infections or malignancy. However, not every... (Review)
Review
Pulmonary opacities/nodules are common findings on computed tomography examinations, which may represent an underlying infections or malignancy. However, not every pulmonary nodule or opacity represents malignancy or infection. We present a pictorial essay illustrating common as well as obscure noninfectious, nonmalignant pulmonary lesions. Lesions discussed include organizing pneumonia, Langerhans cell histiocytosis, pulmonary amyloidosis, hyalinizing granuloma, tumourlet (benign localized neuroendocrine cell proliferations), atypical alveolar hyperplasia, inflammatory myofibroblastic tumour, papillary alveolar adenoma, plasma cell granuloma, juvenile xanthogranuloma, and sclerosing hemangiomas. We discuss the clinical presentation, prevalence, radiographic clues, pathology, and diagnostic pitfalls of these rare lesions.
Topics: Humans; Lung; Lung Diseases; Tomography, X-Ray Computed
PubMed: 26690551
DOI: 10.1016/j.carj.2015.07.004 -
Ocular Oncology and Pathology Sep 2018Juvenile xanthogranuloma (JXG) is a rare and benign tumor in infants. A solitary lesion on the eyelid has been reported in patients with JXG. We report a 15-year-old boy...
Juvenile xanthogranuloma (JXG) is a rare and benign tumor in infants. A solitary lesion on the eyelid has been reported in patients with JXG. We report a 15-year-old boy with multiple involvement of JXG on both eyelids. A mass on the left inner canthus was resected because of disturbance of the visual field and a risk of malignancy in terms of central ulceration in the lesion. The mass was examined by light microscopy. The mass had Touton giant cells with a wreath of nuclei surrounded by foamy histiocytes. No malignancy was observed. The mass showed no recurrence after resection.
PubMed: 30320084
DOI: 10.1159/000478101 -
JAAD Case Reports Mar 2021
PubMed: 33598518
DOI: 10.1016/j.jdcr.2020.12.022 -
Ear, Nose, & Throat Journal Jul 2022
PubMed: 35791499
DOI: 10.1177/01455613221113795 -
Frontiers in Medicine 2020Several dermoscopic features of juvenile xanthogranuloma (JXG) have been previously described in single cases or small case series and need to be further verified in a...
Several dermoscopic features of juvenile xanthogranuloma (JXG) have been previously described in single cases or small case series and need to be further verified in a large sample. We aimed to investigate the dermoscopic patterns of JXG in a large case series and the correlations of these with clinical features of different histopathological subtypes of JXG. Patients who underwent dermoscopic evaluation and had a histopathological diagnosis of JXG were recruited. Histological findings, including stage and Ki67 proliferative index and the dermoscopic features of each lesion were recorded. Forty-one patients with JXG were included. The male to female ratio was 1.28: 1 and the median age of onset was 11 months (range: 0-95 months). Fourteen lesions were histologically categorized in the early stage, 17 in the developed stage, and 10 in the late stage. The "setting sun" pattern was observed in 35 lesions (85.4%) and "clouds" of paler yellow areas in 26 lesions (63.4%). The frequency of the "setting sun" pattern was higher in the early and developed stages (30/31) than in the late stage (5/10) ( = 0.002), while that of "clouds" of paler yellow areas was not significantly different between each stage. Branched linear vessels were detected in the early (11/14) and developed stage (6/17), but not in the late stage. The mean Ki67 index of the lesions with linear vessels was 11.8% (range: 2-40%), which was higher than that of lesions without linear vessels (mean index: 5%, range: 1-30%) ( = 0.005). The pigment network and whitish areas were only detected in 6 and 5 lesions in the late stage, respectively. The whitish areas presented either as streak or stellate shape. The pigment network exhibited either in a centric or a peripheral pattern. The "setting sun" pattern is the characteristic dermoscopic features of JXG in the early and developed stages, while whitish areas and pigment network are the characteristic patterns in the late stage. Linear vessels present as branched patterns and mostly occur in the early stage with a high proliferative index, indicating rapid growth. The whitish areas and pigment network may present in various patterns. Dermoscopy is a useful adjunctive tool in the diagnosis and staging of JXG.
PubMed: 33521026
DOI: 10.3389/fmed.2020.618946 -
European Spine Journal : Official... Sep 2008Solitary juvenile xanthogranuloma (JXG) in the spinal column is extremely rare and there has been no report of such a lesion involving C1 and C2 in English literature so... (Review)
Review
Solitary juvenile xanthogranuloma (JXG) in the spinal column is extremely rare and there has been no report of such a lesion involving C1 and C2 in English literature so far. Here, we report and characterize the first case of xanthogranuloma of the upper cervical spine. This case report draws attention to the fact that solitary xanthogranuloma should be considered among possible diagnoses of spinal tumor in children and young adults. An 18-year-old female patient presented to the hospital with intermittent pain in the right side of the neck. MRI studies revealed a huge soft tissue mass to the right side of the C1 and C2 vertebras, and osseous destruction can be found in the cervical spine CT scan. Complete surgical removal of the tumor and occipital-cervical instrumentation with autogenous bone graft were performed with no complications. The patient was free of pain immediately after the surgery with intact neurological functions. Follow-up MRI 6 and 12 months after the surgery showed no residue or recurrence of the tumor. Our report and the literature review indicate that isolated JXG does not show any predilections of localization inside the central nervous system. So a solitary xanthogranuloma should be considered among possible diagnoses of spinal tumor in susceptible patients. Localized JXG shows isointense signals in MRI and enhances homogeneously with gadolinium. Immunohistochemical studies can ensure the diagnosis. Whenever possible, total surgical removal alone seems to be curative.
Topics: Adolescent; Axis, Cervical Vertebra; Bone Transplantation; Cervical Atlas; Female; Humans; Internal Fixators; Magnetic Resonance Imaging; Neck Pain; Neurosurgical Procedures; Plastic Surgery Procedures; Spinal Diseases; Spinal Fusion; Tomography, X-Ray Computed; Treatment Outcome; Xanthogranuloma, Juvenile
PubMed: 18228052
DOI: 10.1007/s00586-008-0606-0 -
Cureus Jun 2019Juvenile xanthogranuloma (JXG) is an uncommon condition affecting the eye. We herein report a rare case of eyelid swelling in paediatric age group. A three-year-old...
Juvenile xanthogranuloma (JXG) is an uncommon condition affecting the eye. We herein report a rare case of eyelid swelling in paediatric age group. A three-year-old Malay boy presented with chronic painless left upper eyelid mass which did not resolve with topical steroid. Clinically, the mass was a non-tender and firm nodular swelling which located at the lateral 1/3 of the left upper lid. Total excisional biopsy of the swelling was done and histopathological findings were consistent with JXG. The systemic associations and the treatment options for ocular JXG are discussed.
PubMed: 31497438
DOI: 10.7759/cureus.5008