Congenital‐type juvenile xanthogranuloma: A case series and literature review

Oza, Vikash S.; Stringer, Thomas; Campbell, Caren; Hinds, Brian; Chamlin, Sarah L.; Frieden, Ilona J.; Shah, Sonal D.

doi:10.1111/pde.13544

Cited by 27 publications

(27 citation statements)

References 50 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In the Kiel Paediatric Tumor Registry spanning 35 years JXG accounted for 129 (0.5%) out of 24.600 paediatric lesions. It is predominantly a disease of infancy or early childhood with a median age of onset between 5 months and 1 year [5], but congenital-type juvenile xanthogranuloma is also reported [6]. More males are affected than females, with a ratio of 1.4:1.…”

Section: Introductionmentioning

confidence: 99%

The various clinical spectra of juvenile xanthogranuloma: imaging for two case reports and review of the literature

et al. 2019

View full text Add to dashboard Cite

Background Juvenile xanthogranuloma (JXG) belongs to the heterogeneous group of non-Langerhans cell histiocytosis and is caused by an accumulation and proliferation of macrophages. In the majority of cases JXG is a disorder of early childhood presenting during the first 2 years of life. The typical presentation is a solitary reddish or yellowish skin papule or nodule with spontaneous regression and no need for treatment. Case presentation Two infants with an atypical presentation of JXG, one with multiple blueberry muffin rash-like skin lesions and the other with severe multi-systemic involvement, are reported. Diagnosis was established by skin biopsy including histological work-up and immunostaining, where markers for macrophages (CD68 and CD163) exhibited significant reactivity. Conclusion JXG is the most common of the non-Langerhans cell histiocytosis. The typical presentation is a solitary skin lesion. The purpose of this report is to familiarize paediatricians with an unusual variant of this entity in order to facilitate early diagnosis and raise awareness for possible visceral complications and associated medical conditions.

show abstract

Section: Introductionmentioning

confidence: 99%

The various clinical spectra of juvenile xanthogranuloma: imaging for two case reports and review of the literature

et al. 2019

View full text Add to dashboard Cite

show abstract

“…SJXG usually develops in patients with multiple skin lesions and is more common in congenital than acquired JXG 4 . Oza et al reported systemic involvement in 16 of 47 congenital JXG patients 5 . Usually, systemic involvement occurs after cutaneous involvement, as in our patient.…”

Section: Discussionmentioning

confidence: 49%

“…If there is cytopenia, bone marrow aspiration can be performed. The eye is another important site of extracutaneous involvement, especially in patients with multiple cutaneous lesions, so ophthalmologic evaluation is necessary for all patients 2,5 …”

Section: Discussionmentioning

confidence: 99%

Successful treatment of congenital systemic juvenile xanthogranulomatosis with pulmonary involvement

Ataseven

Kızmazoğlu

Öniz

2021

Pediatric Dermatology

View full text Add to dashboard Cite

Juvenile xanthogranuloma (JXG) is a common form of non-Langerhans cell histiocytosis, which usually presents with spontaneously regressing skin lesions. Systemic involvement is rare and mostly seen in patients with multiple skin nodules. It can spontaneously regress, but sometimes systemic involvement can cause life-threatening symptoms and can be fatal. Herein, we report a case of congenital systemic JXG with multiple skin nodules, soft tissue and pulmonary involvement. She was successfully treated with chemotherapy according to Langerhans cell histiocytosis treatment protocol IV of the Histiocyte Society (LCH-IV).

show abstract

“…Congenital JXG is the rarest type of JXG lesions. A meta‐analysis conducted by Oza et al 18 . reports that the behavior of congenital JXG resembles the typical JXG lesions, although authors mentioned that it should be emphasized that infants with multiple congenital JXG lesions should be evaluated in terms of hepatic involvement.…”

Section: Discussionmentioning

confidence: 99%

Juvenile xanthogranuloma: retrospective analysis of 44 pediatric cases (single tertiary care center experience)

Kundak¹,

Çakır

2020

Int J Dermatology

View full text Add to dashboard Cite

Background/Objective Juvenile xanthogranuloma (JXG) is a rarely encountered skin disorder, which is characterized by the proliferation of non‐Langerhans cell histiocytes. As JXG primarily affects infants and young children, this study aims to describe the epidemiologic, clinical, and histopathologic characteristics of 44 children diagnosed with JXG at a tertiary health care center. Methods Fourty‐four children with a histopathologic diagnosis of JXG between January 2003 and January 2017 were retrospectively reviewed. Data related to epidemiologic, clinical, and histopathologic features were extracted from hospital records. Results The mean age of the affected patients was 4.6 years old (range: 0–17 years old) at the time of diagnosis. Twenty‐five patients (56.8%) were male, and 19 patients were female (43.2%). Thirty‐six children (81.8%) had solitary JXG, one of which was a giant congenital JXG; eight children (18.2%) had eruptive JXG. The heterozygote mutation associated with neurofibromatosis 1 gene was detected in one patient who had both eruptive JXG and numerous café‐au‐lait spots. Another patient with eruptive JXG was identified to have hypercholesterolemia. None of the children with eruptive JXG developed symptoms or signs of extracutaneous involvement during their clinical follow‐up. Conclusion Since JXG is rarely encountered, there may be a tendency toward over‐treatment, given concerns for extracutaneous involvement. However, our review revealed no instances of extracutaneous involvement.

show abstract

Congenital‐type juvenile xanthogranuloma: A case series and literature review

Cited by 27 publications

References 50 publications

The various clinical spectra of juvenile xanthogranuloma: imaging for two case reports and review of the literature

The various clinical spectra of juvenile xanthogranuloma: imaging for two case reports and review of the literature

Successful treatment of congenital systemic juvenile xanthogranulomatosis with pulmonary involvement

Juvenile xanthogranuloma: retrospective analysis of 44 pediatric cases (single tertiary care center experience)

Contact Info

Product

Resources

About