Its most common site of extracutaneous presentation is the eyes. [pathologyoutlines.com]

Although this benign disorder typically presents with a favorable clinical outcome, the unusual presentation as an intratesticular lesion can lead to diagnostic challenges. [ncbi.nlm.nih.gov]

Ocular manifestations usually present in the form of iris lesions with secondary hyphema and glaucoma, but juvenile xanthogranuloma may also present as a corneoscleral limbal mass. [thedoctorsdoctor.com]

We report the use of dermoscopy (“setting sun” pattern) as an adjuvant tool in the diagnosis of juvenile xanthogranuloma in a female patient presenting with a 2-month history of a pre-auricular papule. [anaisdedermatologia.org.br]

• Swelling

  To report the clinical and pathologic features of an elderly patient with a unilateral orbital swelling and proptosis caused by Juvenile Xanthogranuloma diagnosed and confirmed by orbital biopsy. Interventional case report. One patient. [ncbi.nlm.nih.gov]

  A child of ten months age reported to the Azeezia College of Dental Sciences and Research with a nodular swelling on the right side of the cheek and gave a history of swelling since the age of 5 months with gradual increase in size which was not associated [scielo.conicyt.cl]

  Chan, MD IMPORTANCE Juvenile recurrent parotitis (JRP) is characterized by recurrent painful swelling of the parotid gland that occurs in the pediatric population. [docksci.com]

• Heterochromia Iridis

  Ocular JXG is usually unilateral and presents with a tumor, a red eye with signs of uveitis, unilateral glaucoma, spontaneous hyphema or heterochromia iridis. [en.wikipedia.org]

  Eye involvement is usually but not always unilateral, and commonly presents with an asymptomatic iris tumour, a red eye with signs of uveitis, unilateral glaucoma, spontaneous hyphema or heterochromia iridis (3). [ncbi.nlm.nih.gov]

  Eye involvement is usually, but not always, unilateral and commonly presents with an asymptomatic iris tumour, a red eye with signs of uveitis, unilateral glaucoma, spontaneous hyphema, or heterochromia iridis [6]. [karger.com]

• Papule

  Comment Here Reference: Juvenile xanthogranuloma Board review style question #2 A 2 year old girl was brought to the clinic for an asymptomatic red-yellow papule of a few months' duration on the arm. [pathologyoutlines.com]

  […] tho-gran″u-lo´mah ] a tumor having histologic characteristics of both granuloma and xanthoma. juvenile xanthogranuloma a benign, self-limited disorder of infants and children, usually present at birth, manifested by the development of single or multiple papules [medical-dictionary.thefreedictionary.com]

  It manifests clinically as a red-yellow papule, usually showing spontaneous remission. The diagnosis is based on clinical and histological findings. [anaisdedermatologia.org.br]

  This is a case of a 19-year-old woman who presented with progressive development of tan-red papules on the axilla and eyelids over a 1.5-year time span. [iths.pure.elsevier.com]

• Xanthoma

  […] multiplex, juvenile xanthoma, multiple eruptive xanthoma in infancy, congenital xanthoma tuberosum, xanthoma neviforme and juvenile giant cell granuloma ICD coding ICD-10: D76.3 - Other histiocytosis syndromes Epidemiology Incidence is unknown Between [pathologyoutlines.com]

  xanthoma tuberosum, xanthoma naeviforme and juvenile giant-cell granuloma. [dermnetnz.org]

  Xanthoma disseminatum is also a non-X histiocytosis, but lacks foreign body-type giant cells and eosinophils both pf which are usually present in juvenile xanthogranuloma. [path.upmc.edu]

  Xanthoma neviforme JXG Statements Identifiers Sitelinks Wikipedia (4 entries) edit Wikibooks (0 entries) edit Wikiquote (0 entries) edit Wikisource (0 entries) edit Wikiversity (0 entries) edit Wikivoyage (0 entries) edit Wiktionary (0 entries) edit [wikidata.org]

• Ulcer

  We report a case of ulcerated atypical Spitz nevi that demonstrated a yellow to light orange background under dermoscopy, which can be seen in juvenile xanthogranuloma (JXG) and is referred to as the "setting sun" appearance. [ncbi.nlm.nih.gov]

• Hyperpigmentation

  […] males In neonates, rarely associated with giant cell hepatitis and tumor in liver and viscera, requiring chemotherapy Treatment Conservative excision ( Am J Surg Pathol 2005;29:21 ) May spontaneously regress leaving depressed area of skin with variable hyperpigmentation [pathologyoutlines.com]

  In infancy, there is a male predominance in a ratio of 1.5:1, with a tendency to spontaneous regression, which may result in atrophy and hyperpigmentation.1,3,4 Although the term juvenile xanthogranuloma is used, adult cases can occur, especially in patients [anaisdedermatologia.org.br]

  It is worth to note that regression is capable of generating atrophy and hyperpigmentation.1 Dermoscopy is useful in assisting the diagnosis, since this condition has a typical pattern, characterized by an orange color background – described as the "setting [surgicalcosmetic.org.br]

  Histopathological examination of JXG with whitish area revealed dermal fibrous tissue, while the pigment network represents hyperpigmentation in the basement of the epidermis. Figure 1. [frontiersin.org]

  Residual atrophy, anetoderma, or hyperpigmentation are not uncommon after lesions resolve. Expected results of diagnostic studies There is no dyslipidema associated with JXGs. There are no known associated laboratory anomalies. [dermatologyadvisor.com]

• Skin Disease

  Juvenile Xanthogranuloma (JXG) is the most common Non-X Histiocytosis and I occasionally encounter this skin disease in both my The Woodlands dermatology and Conroe dermatology offices. [perridermatology.com]

  Skin involvement in other diseases. [pascal-francis.inist.fr]

  Diseases of the Digestive Tract Endocrine Disorders Hematopoesis Immunologic Diseases Infections Mesenchyme and Skeleton Metabolic Diseases Normalbefunde, Visusminderung bei R/L Astigmatismus Phakomatoses Pseudoexfoliation Syndrome (PEX) Skin Cataracta [atlasofophthalmology.com]

  Disease Clinical apps Boards fodder archive Choosing Wisely Appropriate use Industry showcase webinars Featured Clinical guidelines Review current clinical guidelines, those in development, and guidelines that the AAD has collaborated on. [aad.org]

  Since the 1980s, dermoscopy has become widely used in the diagnosis of pigmented lesions and has been gaining popularity for its ability to differentiate between nonpigmented skin tumors and inflammatory skin diseases. [frontiersin.org]

• Neck Mass

  We report a rare case of juvenile xanthogranuloma in a 16-year-old girl who presented with a neck mass and left-sided ptosis 2.5 months previously. [ncbi.nlm.nih.gov]

  Awareness of these features should prompt radiologists to include juvenile xanthogranuloma in the differential diagnosis of pediatric head and neck masses. [ajnr.org]

  Juvenile xanthogranuloma presenting as a large neck mass and ocular complications: a diagnostic and therapeutic dilemma. BMJ Case Rep. 2014. doi:10.1136/bcr-2013-202683. 10. Pajaziti L, Hapciu SR, Pajaziti A. [consultant360.com]

These data highlight the importance of identifying specific MAPK pathway alterations as part of the diagnostic workup for patients with histiocytic disorders rather than initiating empiric treatment with MEK inhibitors. [ncbi.nlm.nih.gov]

In Neurofibromatosis 1, JXGs are also more prevalent so workup for this genetic disease may be initiated with the discovery of JXG. [perridermatology.com]

Results from a medical workup, which included computed tomography of the orbits, were unremarkable. Evaluation of the patient's skin revealed several yellow-orange to yellow-brown pigmented nodular lesions on the face and scalp. [jamanetwork.com]

Laboratory tests and autoimmune workup were completed in 7 patients. [docksci.com]

However, this case provides an example in which MEK inhibition would presumably be ineffective, supporting a strategy of identifying the specific MAPK pathway alterations in each case as part of the diagnostic workup for patients with histiocytic disorders [oncotarget.com]

• Lymphocytic Infiltrate

  In addition, in the upper and lower dermis, there were giant histiocytes, mainly of Touton type, as well as some foamy histiocytes with mixed lymphocytic infiltrations, which was extended to subcutaneous tissue (Figure 2). [dovepress.com]

  infiltrate and dermal fibrosis.[9] Although positive for CD68, these are negative for vimentin (unlike juvenile xanthogranuloma).[9] Although juvenile xanthogranulomas may be found in uncommon locations (i.e., vulva), it is important to remember that [ncbi.nlm.nih.gov]

• Foam Cell

  […] hypercholesterolemia and high LDL Consists of dermal aggregates of foam cells, no Touton cells or inflammatory cells Spitz nevus: Nests of spindle and epithelioid cells within a uniformly hyperplastic epidermis Melanocytic immunostaining with S100, MelanA [pathologyoutlines.com]

  Numerous foam cells are seen throughout the dermis (original magnification × 4) (A). Giant cells and numerous foam cells are seen. Arrows indicate Touton giant cells (original magnification × 20) (B). Fig. 3. [medicaljournals.se]

  Papular xanthomas contain foam cells and Touton giant cells, but lack the mixed inflammatory infiltrate found in juvenile xanthogranulomas. Other considerations include spitz nevus and reticulohistiocytoma. REFERENCES: Adamson, HG. [path.upmc.edu]

  […] histiocytosis.[4] Can rarely be found in the brain.[5] Microscopic Features:[4] Dermal histiocytes: Abundant cytoplasm - may not be xanthomatous/foam cells. [librepathology.org]

The initial neurological symptoms resolved without any surgical or medical treatment. [ncbi.nlm.nih.gov]

Self limited No treatment is usually required Excision can be done for cosmetic concerns Reference: Actas Dermosifiliogr 2020;111:725 Clinical images Contributed by Lamiaa Hamie, M.D., M.Sc. and Ossama Abbas, M.D.   [pathologyoutlines.com]

Although intracranial involvement is rare, it differs from other systemic JXG because its responsiveness to non-surgical treatment is poor. We present the case of a 2-year-old boy with left abducens nerve palsy. [doi.org]

The patient was thus expected with benign prognosis, and additional resection of the tumor including vocal fold was not indicated in the initial treatment. Six weeks later, the JXG recurred and a second procedure using CO₂ laser was needed. [ncbi.nlm.nih.gov]

Juvenile xanthogranuloma juvenile xanthogranuloma Juvenile lipogranuloma, nevoxanthoendothelioma A yellowish tumor of early childhood, involving the face, head, neck, extremities Prognosis Spontaneous involution xanthogranuloma, juvenile (JXG) A benign [medical-dictionary.thefreedictionary.com]

In patients with other ocular or orbital involvement, prognosis varies with the extent of the disease and its response to treatment. [emedicine.com]

The etiology of juvenile xanthogranuloma is uncertain, although the occurrence in monozygotic twins may suggest genetic predisposition. © 2010 Wiley Periodicals, Inc. [ncbi.nlm.nih.gov]

The exact etiology is unclear, but delayed-type hypersensitivity may be implicated in the pathogenesis. Complete surgical excision appears to be curative. No recurrences have been reported. [humpath.com]

J Otol Rhinol 6:3. doi: 10.4172/2324-8785.1000313 Abstract Juvenile xanthogranuloma (JXG) is a benign self-limiting non langerhans cell histiocytic lesion of unknown etiology and pathogenesis. [scitechnol.com]

[…] and most often develops before the age of 2 Hyphema and glaucoma are serious complications of ocular JXG Systemic involvement is rare, occurring in less than 5% of cases Reference: Actas Dermosifiliogr 2020;111:725 Pathophysiology Unknown at this time Etiology [pathologyoutlines.com]

Epidemiology This is predominantly a disorder of the first two decades of life, 45% 2 to 75% 3 of patients developing the lesions as infants, 95% by the age of 10 years 3. [e-immunohistochemistry.info]

Epidemiology Uncommon (< 0.5% of pediatric tumors in one study) Usually infants (median age 5 months) with a congenital mark, although 10 - 30% occur in adults Male / female = 1.4 : 1 Clinical features Skin of face or trunk, less commonly in subcutis, [pathologyoutlines.com]

The epidemiology, clinical characteristics, histopathology and management of juvenile- and adult-onset corneoscleral limbus xanthogranuloma. Graefes Arch Clin Exp Ophthalmol. 2016 Mar. 254 (3):413-20. [Medline]. [emedicine.com]

But due to rarity of non Langerhans cell hystiosis itself, the exact prevalence of juvenile xanthogranuloma remain unknown with only a few epidemiological journal ever published. [bioscmed.com]

[…] organs Ocular JXG is usually unilateral and most often develops before the age of 2 Hyphema and glaucoma are serious complications of ocular JXG Systemic involvement is rare, occurring in less than 5% of cases Reference: Actas Dermosifiliogr 2020;111:725 Pathophysiology [pathologyoutlines.com]

Etiology and Pathophysiology The pathogenesis is unknown. [accesspediatrics.mhmedical.com]

Pathophysiology The etiology of JXG is unknown. JXG is believed to result from a disordered macrophage response to a nonspecific tissue injury, resulting in a granulomatous reaction. [emedicine.com]

Review the pathophysiology of xanthogranulomas. Explain interprofessional team strategies for improving care coordination and communication regarding the management of patients with xanthogranulomas. [ncbi.nlm.nih.gov]

We performed extended excision to prevent recurrence and the tumor did not recur after resection. Keywords juvenile xanthogranuloma histiocytosis Touton giant cell [iranjd.ir]

Eye involvement with JXG needs to be treated to prevent visual problems. Systemic JXG, where lesions are present in more than one organ or body system, has been reported only in single- or small-case studies and usually requires treatment. [histio.org]

Solu Decortin H) 10 mg/day p.o., especially in case of eye involvement to prevent glaucoma or loss of vision. Progression/forecast This section has been translated automatically. Spontaneous regression between the ages of 1 and 6. [altmeyers.org]