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Diagnostic Cytopathology Jan 2020Juvenile xanthogranuloma (JXG) is a rare type of non-Langerhan cell histiocytic disorder, which is mostly confined to skin of head and neck. It is a self-limiting benign...
Juvenile xanthogranuloma (JXG) is a rare type of non-Langerhan cell histiocytic disorder, which is mostly confined to skin of head and neck. It is a self-limiting benign condition, which does not require surgery. We present a case of 8-month-old girl child with multiple yellowish brown colored papules over scalp, face, and neck. A clinical diagnosis of cutaneous mastocytosis was made. Fine-needle aspiration cytology (FNAC) smears showed foamy macrophages along with mixed inflammatory infiltrate and few touton giant cells. A diagnosis of JXG was rendered which was confirmed on histopathology and immunohistochemistry.Juvenile xanthogranuloma can be diagnosed on FNAC based on its characteristic cytologic features; however, it requires a high index of suspicion by cytopathologist. Cytological diagnosis of JXG can save the patient from unnecessary surgical biopsy or excision.
Topics: Biopsy, Fine-Needle; Female; Histiocytes; Humans; Infant; Skin; Xanthogranuloma, Juvenile
PubMed: 31432610
DOI: 10.1002/dc.24310 -
Archives of Disease in Childhood Apr 2013
Topics: Diagnosis, Differential; Face; Female; Humans; Infant; Xanthogranuloma, Juvenile
PubMed: 23235892
DOI: 10.1136/archdischild-2012-303338 -
Pediatric Dermatology 2010Juvenile xanthogranuloma is usually a benign condition mainly seen in infants and children. It frequently presents as asymptomatic discrete papules on the head, trunk,...
Juvenile xanthogranuloma is usually a benign condition mainly seen in infants and children. It frequently presents as asymptomatic discrete papules on the head, trunk, and limbs. Extracutaneous manifestations, most commonly ocular, are rare but may be associated with significant morbidity. The etiology of juvenile xanthogranuloma is uncertain, although the occurrence in monozygotic twins may suggest genetic predisposition.
Topics: Humans; Infant; Scalp; Twins, Monozygotic; Xanthogranuloma, Juvenile
PubMed: 21091658
DOI: 10.1111/j.1525-1470.2010.01332.x -
BMJ Case Reports Mar 2021
Topics: Child, Preschool; Humans; Xanthogranuloma, Juvenile
PubMed: 33771776
DOI: 10.1136/bcr-2020-241411 -
Pediatric Dermatology Jul 2020Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis characterized by yellowish papules in the skin. JXGs most often occur in infancy or early childhood... (Review)
Review
BACKGROUND
Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis characterized by yellowish papules in the skin. JXGs most often occur in infancy or early childhood and are typically solitary and asymptomatic, often regressing after several years. While JXGs predominantly occur on the skin, extracutaneous JXGs also exist.
AIMS
In this paper, we review the literature on single, multiple, and visceral JXGs and provide recommendations on monitoring and work-up.
MATERIALS & METHODS
A literature review was conducted with the PubMed database using selective search terms for single, multiple, ocular, and visceral lesions as well as NF1/JMML.
RESULTS / DISCUSSION
JXG is typically a self-limited disorder if lesions are cutaneous and singular. While rare, JXGs may manifest as multiple and extracutaneous lesions. Further screening and referral to specialists may be warranted in these cases based on age and extent of involvement.
CONCLUSION
Our review demonstrates common presentations of single, multiple, and extracutaneous lesions in addition to those that occur with NF1 and JMML. We suggest patients be evaluated on a case-by-case basis by a dermatologist and referred to specialists as appropriate.
Topics: Child, Preschool; Histiocytosis, Non-Langerhans-Cell; Humans; Infant; Skin; Xanthogranuloma, Juvenile
PubMed: 32468628
DOI: 10.1111/pde.14174 -
Archives of Pathology & Laboratory... Sep 1989The changing name of the juvenile xanthogranuloma bears witness to the evolution of knowledge and experience of its varied clinical and histologic presentations. This...
The changing name of the juvenile xanthogranuloma bears witness to the evolution of knowledge and experience of its varied clinical and histologic presentations. This study characterizes the clinical, microscopic, and some immunohistochemical features of 34 cases. The salient clinical findings include a bimodal age distribution inclusive of adults, a male:female ratio of 4:1, occasional multiplicity of lesions (20%), and common presentation in the cephalad area. Histologic findings include varied architectural patterns, cellular participation in various proportions by foamy histiocytes, epithelioid monocytes, lymphocytes, plasma cells, eosinophils, Touton giant cells, and spindle cells of two forms (dendritic and fusiform). S100-positive dendritic cells comprised a minor, but important, component at expansion zones. The significance of these findings is described.
Topics: Adolescent; Adult; Age Factors; Child; Child, Preschool; Epidermis; Female; Humans; Infant; Infant, Newborn; Male; Middle Aged; S100 Proteins; Xanthogranuloma, Juvenile
PubMed: 2505733
DOI: No ID Found -
Journal of the European Academy of... Nov 2022
Topics: Dermoscopy; Humans; Skin; Skin Pigmentation; Xanthogranuloma, Juvenile
PubMed: 35691005
DOI: 10.1111/jdv.18319 -
Journal of Hand Surgery (Edinburgh,... Oct 1984A case of juvenile xanthogranuloma in the hand is reported. The lesion was atypical clinically, being sited in the hand and the tumour was so large that it extended from...
A case of juvenile xanthogranuloma in the hand is reported. The lesion was atypical clinically, being sited in the hand and the tumour was so large that it extended from the palmar to the dorsal surface. Curettage was performed at seven weeks after birth and histological examination established a diagnosis of juvenile xanthogranuloma. The post-operative course was uneventful, and the residual lesion had disappeared spontaneously four years after operation.
Topics: Female; Follow-Up Studies; Hand; Humans; Infant; Wound Healing; Xanthogranuloma, Juvenile
PubMed: 6439806
DOI: 10.1016/0266-7681(84)90059-7 -
Actas Dermo-sifiliograficas Sep 2017
Topics: Child; Female; Humans; Ultrasonography; Xanthogranuloma, Juvenile
PubMed: 28372783
DOI: 10.1016/j.ad.2016.11.021 -
Pediatrics in Review Oct 2022Histiocytic disorders of childhood represent a wide spectrum of conditions that share the common histologic feature of activated or transformed "histiocytes." Langerhans...
Histiocytic disorders of childhood represent a wide spectrum of conditions that share the common histologic feature of activated or transformed "histiocytes." Langerhans cell histiocytosis (LCH) is the most common, with an incidence of approximately 5 per million children. LCH may be difficult to distinguish from more ubiquitous causes of skin rashes, bone pain, or fever. Current chemotherapy fails to cure more than 50% of children with multifocal disease, and treatment failure is associated with increased risks of long-term sequelae. Somatic activating mitogen-activated protein kinase (MAPK) pathway-activating mutations (most often BRAFV600E) have been identified in hematopoietic precursors in patients with LCH. Opportunities to improve outcomes with targeted therapies are under investigation. Juvenile xanthogranuloma (JXG) and Rosai-Dorfman disease (RDD) are less common than LCH and are distinguished by specific histologic and clinical features. Recurrent MAPK pathway gene mutations are also identified in JXG and RDD. In many cases, these conditions spontaneously resolve, but disseminated disease can be fatal. Although there has been historic debate regarding the nature of these conditions as inflammatory versus neoplastic, LCH, JXG, and RDD are now considered myeloid neoplastic disorders. In contrast, hemophagocytic lymphohistiocytosis (HLH) is clearly a disorder of immune dysregulation. HLH is characterized by extreme immune activation driven by hyperactivated T cells. HLH arises in approximately 1 child per million and is nearly universally fatal without prompt recognition and immune suppression. Outcomes of treated children are poor, with approximately 60% survival. Emapalumab, which targets interferon-γ signaling, was recently approved for patients with recurrent or refractory HLH, and additional cytokine-directed therapies are under investigation.
Topics: Child; Histiocytes; Histiocytosis, Langerhans-Cell; Histiocytosis, Sinus; Humans; Interferon-gamma; Mitogen-Activated Protein Kinases; Xanthogranuloma, Juvenile
PubMed: 36180546
DOI: 10.1542/pir.2021-005367