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Journal of the European Academy of... Nov 2022
Topics: Dermoscopy; Humans; Skin; Skin Pigmentation; Xanthogranuloma, Juvenile
PubMed: 35691005
DOI: 10.1111/jdv.18319 -
Clinical and Experimental Dermatology Jan 2022Non-Langerhans cell histiocytosis is a collective term encompassing a vast group of benign proliferative disorders of histiocytes, macrophages and dendritic cells that...
Non-Langerhans cell histiocytosis is a collective term encompassing a vast group of benign proliferative disorders of histiocytes, macrophages and dendritic cells that do not meet the criteria of Langerhans cell histiocytosis. We describe a case of juvenile xanthogranuloma with an unusual clustered distribution.
Topics: Dermoscopy; Diagnosis, Differential; Female; Humans; Infant; Thorax; Xanthogranuloma, Juvenile
PubMed: 34585771
DOI: 10.1111/ced.14912 -
Neurology India 2023
Topics: Humans; Xanthogranuloma, Juvenile; Cognitive Dysfunction
PubMed: 36861607
DOI: 10.4103/0028-3886.370469 -
Head and Neck Pathology Jun 2022Juvenile xanthogranuloma (JXG) is the most common form of non-Langerhans cell histiocytosis and oral mucosal involvement is exceedingly rare. Histiocytic disorders... (Review)
Review
Juvenile xanthogranuloma (JXG) is the most common form of non-Langerhans cell histiocytosis and oral mucosal involvement is exceedingly rare. Histiocytic disorders harbor activating mutations in MAPK pathway, including the report of BRAF V600E in JXG of extracutaneous site. However, no information is available for oral JXG. Herein, the clinicopathological and immunohistochemical features of five new oral JXG were evaluated in conjunction with literature review. Also, we assessed the BRAF V600E in oral samples. Five oral JXG were retrieved from pathology archives. Morphological and immunohistochemical analyses were performed. The BRAF V600E status was determined with TaqMan allele-specific qPCR. The series comprised of three female and two male patients, most of them adults, with a median age of 39 years (range 13-68 years). Clinically, the lesions appeared as asymptomatic solitary nodules, measuring until 2.5 cm, with more incident to the buccal mucosa. Morphologically, most of the cases presented classical histological features of JXG, with histiocytic cells consistent with the non-Langerhans cell immunophenotype. BRAF V600E was not detected in the cases tested. This is the first and largest published series of oral JXG affecting adults and a Brazilian population. The molecular pathogenesis of oral JXG remains unknown. Clinicians and pathologists must recognize JXG to avoid misdiagnoses with oral benign or malignant lesions.
Topics: Adolescent; Adult; Aged; Brazil; Female; Humans; Male; Middle Aged; Mutation; Proto-Oncogene Proteins B-raf; Xanthogranuloma, Juvenile; Young Adult
PubMed: 34414559
DOI: 10.1007/s12105-021-01373-x -
Skin Research and Technology : Official... Sep 2022Molluscum contagiosum (MC), milia, keratosis pilaris (KP), verruca plana (VP), seborrheic keratosis (SK), and juvenile xanthogranuloma (JXG) are common papule dermatoses...
BACKGROUND
Molluscum contagiosum (MC), milia, keratosis pilaris (KP), verruca plana (VP), seborrheic keratosis (SK), and juvenile xanthogranuloma (JXG) are common papule dermatoses on the face of children that have a similar appearance. In vivo evaluation of facial papule dermatoses with reflectance confocal microscopy (RCM) is helpful in the diagnosis of these ambiguous lesions in children. The purpose of this study was to clarify the RCM characteristics of MC, milia, KP, VP, SK, and JXG and explore the clinical application value of RCM for these common facial papule dermatoses.
METHODS
We recruited 113 patients referred for unequivocal facial papule dermatosis, including 21 patients with MC, 17 patients with milia, 19 patients with KP, 36 patients with VP, 8 patients with SK, and 12 patients with JXG. We evaluated the characteristics and distinguishing features of the six kinds of facial papule dermatoses using RCM.
RESULTS
The main RCM features of the six dermatoses included a well-demarcated border of the lesion area. MC, milia and KP all manifested cyst-like structures, and their distinguishing features were the location of the cystic structures and the refractive index of the contents. Although VP, SK, and JXG did not have obvious cystoid structures, VP was typically characterized by uniformly distributed petal-like structures with a medium-to-high refractive index in the epidermis. With regard to SK, the characteristic features were an obviously thickened epidermis and cobblestone-like structures. JXG was mainly characterized by multiple large round and ovoid cells with a foamy cytoplasm, and discoid-shaped multinucleated large cells were diffusely distributed in the dermis.
CONCLUSION
RCM allows the real-time visualization of major key diagnostic and distinguishing features of common facial papule dermatoses in children, including MC, milia, KP, VP, SK, and JXG.
Topics: Child; Dermoscopy; Diagnosis, Differential; Facial Dermatoses; Humans; Keratosis, Seborrheic; Microscopy, Confocal; Skin Neoplasms; Warts
PubMed: 35726961
DOI: 10.1111/srt.13170 -
Pediatric Neurosurgery 2021Juvenile xanthogranuloma (JXG) is a disorder of histiocytic proliferation that affects young children and usually presents as spontaneously regressing cutaneous lesions....
INTRODUCTION
Juvenile xanthogranuloma (JXG) is a disorder of histiocytic proliferation that affects young children and usually presents as spontaneously regressing cutaneous lesions. JXG with systemic involvement is a rare entity associated with significant morbidity and mortality. Intracranial solitary lesions are uncommon, and when comorbid with multiple lesions of the central nervous system in young children, it has an extremely worse prognosis.
CASE PRESENTATION
We have reported here an unusual case of a 6-year-old boy who initially presented with the complaints of headache, vomiting, seizure, and speech disorder without cutaneous and other organ involvement and a neurological tendency to sleep. Acute hydrocephalus was detected in his brain CT. As an emergency intervention, ventriculo-peritoneal shunt operation was performed on the patient. His postoperative MRI revealed a disseminated intracranial disease involving the extensive dural, sellar-suprasellar region, the orbit, and the brain parenchyma. The patient accordingly underwent a pterional approach for open biopsy and for the mass tissue diagnosis. Histopathology reports were consistent with JXG. Unfortunately, the patient succumbed to the disseminated disease within 2 months of the JXG diagnosis.
CONCLUSION
JXG is a disorder that usually affects the skin. Intracranial lesion can be simple or have multiple involvement. This is a rare case of fatal disseminated multiple intracranial JXG without cutaneous and other organ manifestations. The presentation as a sellar-suprasellar, extensive dural, orbit, and parenchymal involvement at the time of diagnosis is unusual and rarely described in the literature.
Topics: Biopsy; Central Nervous System; Child; Child, Preschool; Humans; Magnetic Resonance Imaging; Male; Seizures; Xanthogranuloma, Juvenile
PubMed: 33735896
DOI: 10.1159/000513940 -
Journal of Cutaneous Medicine and... Nov 2023
Topics: Humans; Cicatrix; Xanthogranuloma, Juvenile; Connective Tissue Diseases; Xanthomatosis
PubMed: 37795963
DOI: 10.1177/12034754231194010 -
JAAD Case Reports Oct 2020
PubMed: 32995422
DOI: 10.1016/j.jdcr.2020.07.034 -
Experimental Oncology Dec 2022The work describes a case of rare neonatal systemic juvenile xanthogranuloma with an initial damage of the scalp, limbs, back and abdomen, multiple damages of...
The work describes a case of rare neonatal systemic juvenile xanthogranuloma with an initial damage of the scalp, limbs, back and abdomen, multiple damages of the parenchyma of both lungs, spleen and liver with the development of a severe form of congenital cholestatic hepatitis. The diagnosis was established on the basis of histopathological and immunohistochemical examination of the skin nodules. The child on the background of therapy under the Langerhans cell histiocytosis III program achieved a partial response, which was manifested by a reduction of granulomatous formations on the skin, elimination of liver failure, but retained hepatosplenomegaly, specific lesions of the lung parenchyma, liver, and left kidney. Against the background of cytostatic therapy, the patient developed secondary pancytopenia, perianal ulcerative-necrotic dermatitis with lesions on buttocks, stomatitis, protein-energy deficiency, acute liver failure. coagulopathy, disseminated intravascular coagulation syndrome, acute renal failure, respiratory failure of III degree, cardiovascular insufficiency of III degree, pulmonary edema, cerebral edema, cerebral coma of II-III degree, enterocolitis, intestinal paresis. Despite multicomponent intensive care, the child's condition progressively deteriorated, and the patient died. The aspects of differential diagnosis of neonatal systemic juvenile xanthogranuloma are discussed.
Topics: Infant, Newborn; Child; Humans; Xanthogranuloma, Juvenile; Skin; Diagnosis, Differential; Pancytopenia; Liver
PubMed: 36811532
DOI: 10.32471/exp-oncology.2312-8852.vol-44-no-4.19193 -
Cureus Aug 2020Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis that typically presents as a solitary lesion in infancy. Multiple lesions, especially in patients...
Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis that typically presents as a solitary lesion in infancy. Multiple lesions, especially in patients over one year of age, are rarely described in the literature. The authors report a case of a 17-year-old female who presented with multiple asymptomatic nodules and plaques. The diagnosis of xanthogranuloma was confirmed with histopathologic examination of foamy histiocytes and the characteristic Touton giant cells. The expected course of multiple JXG in older patients may differ from those presenting with a solitary lesion earlier in life.
PubMed: 32884872
DOI: 10.7759/cureus.9516