Fifty percent of patients never develop skin lesions and may first present to the ophthalmologist.7 Treatment depends on the presenting signs and symptoms. [hkmj.org]

Conclusion To conclude, JXG may infrequently present as an ocular surface mass in children. JXG must be kept in the differential diagnosis especially if there are associated skin lesions or a history of the same is present. [ijoreports.in]

Adult reticulohistiocytosis classically presents as red to yellow-red dermal nodules. [scienceopen.com]

It is present at birth in 5% to 17% of the cases but it mainly arises within the first year of life (4). We present a case report of a baby girl with JXG localized in the upper lip. [ncbi.nlm.nih.gov]

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

• Bone Pain

  pain, exophthalmos, diabetes insipidus CD68+, CD163+, CD1a- foamy histiocytes Touton giant cells also often present Board review style question #1 A 1 year old girl was brought to the clinic for an asymptomatic red-yellow papule on her face with the [pathologyoutlines.com]

• Hemorrhagic Bullae

  Congenital self-healing reticulohistiocytosis presenting with hemorrhagic bullae. J Am Acad Dermatol. 2003;48:S75-7. McNutt NS, Reed JA. Tumors of the fibrous tissue. In : Farmer ER, Hood AF eds. Pathology of the skin; 2nd ed. [jurnal.unbrah.ac.id]

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

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

The evidence in such cases is limited and a diagnostic workup guided by clinical findings would appear to be a reasonable strategy. Treatment tends to involve watchful waiting, although chemotherapy may be required for systemic cases. [actasdermo.org]

The varied diagnostic workup and clinical outcome illustrate difficulties that may be encountered with this entity. [healio.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]

• Ground Glass Appearance

  and in the second place a faint colour. b (HE × 200) High power from area indicated in a nicely outlines xanthomatized cells. c (HE × 200) shows oncocytic/plasmocytoid mononuclear cells with dense amphophilic ground glass cytoplasm, occasional eosinophils [bmcpediatr.biomedcentral.com]

• Chlamydia

  NF1: Neurofibromatosis Type 1 NLD: Non-Langerhans cell disorder PNET: Primitive neuroecodermal tumor RDD: Rosai-Dorfman disease TORCH: Acronym for toxoplasmosis, other (parvovirus B19, varicella zoster virus, listeriosis), rubella, cytomegalovirus, chlamydia [bmcpediatr.biomedcentral.com]

Figure 1 Yellow-brown papulonodular asymptomatic lesions of juvenile xanthogranuloma, at the right leg (A), face (B), right arm (C) before the treatment and left leg (D) after the treatment. [dovepress.com]

Patients with genetic changes or abnormalities (mutations) may benefit more from treatment which targets their tumor's particular genetic mutation, and may help doctors plan better treatment for patients with solid tumors or non-Hodgkin lymphomas. [clinicaltrials.ucsf.edu]

A small percentage of patients with rapidly growing disease may require treatment with chemotherapy or low-dose radiation, however there is no agreed standard. [histiouk.org]

Treatments that have been used include surgical excision, intralesional steroid injection, cryotherapy, and low dose radiotherapy. In the case of a resistant or reoccurring lesion, chemotherapy has been used as a treatment. [en.wikipedia.org]

Topical steroids are useful in treatment of iris lesions. However, steroids alone are insufficient in treatment of ocular surface lesions as reported by various studies and are mostly used as an adjunctive treatment. [ijoreports.in]

The patient's general health is not impaired and, in the absence of associated conditions, the prognosis is excellent. Diagnosis is readily made in typical cases, but may be more difficult in unusual variants. [read.qxmd.com]

Because congenital JXG has an excellent prognosis, insight into unique presentations such as this may provide useful information and avoid unnecessary surgical interventions. [ncbi.nlm.nih.gov]

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

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]

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]

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]

Etiology and Pathophysiology The pathogenesis is unknown. [accesspediatrics.mhmedical.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. [QxMD MEDLINE Link]. [emedicine.medscape.com]

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]

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]

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

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.medscape.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]

A complete excision is usually advocated as it prevents its extension to deep corneal stroma. [ijoreports.in]