-
The Journal of Pediatrics Dec 2022
Topics: Humans; Xanthogranuloma, Juvenile
PubMed: 36030949
DOI: 10.1016/j.jpeds.2022.08.008 -
Disseminated juvenile xanthogranulomas with ocular involvement: A case report and literature review.SAGE Open Medical Case Reports 2022Cutaneous juvenile xanthogranuloma is an uncommon disorder usually arising during infancy. Systemic involvement of juvenile xanthogranuloma remains rare, and there are...
Cutaneous juvenile xanthogranuloma is an uncommon disorder usually arising during infancy. Systemic involvement of juvenile xanthogranuloma remains rare, and there are no published guidelines to date on screening extracutaneous manifestations in these patients. Ocular involvement is the most common extracutaneous manifestation of juvenile xanthogranuloma. We present the case of an infant with disseminated juvenile xanthogranulomas and associated ocular involvement and present a review of literature, focusing on identifying risk factors for ocular and systemic involvement in disseminated cases.
PubMed: 35991957
DOI: 10.1177/2050313X221117693 -
Clinical, Cosmetic and Investigational... 2022Non-Langerhans cell histiocytosis (non-LCH) is a group of diseases characterized by the proliferation of histiocytes in tissues that is excluded from the diagnostic...
Non-Langerhans cell histiocytosis (non-LCH) is a group of diseases characterized by the proliferation of histiocytes in tissues that is excluded from the diagnostic criteria for LCH. Juvenile xanthogranuloma (JXG) and benign cephalic histiocytosis (BCH) are the most common types of cutaneous non-LCH. These two diseases share similarities in both clinical and histological features, therefore, they can be difficult to differentiate. Thorough physical, dermoscopic, and histopathological examinations are required to distinguish between JXG and BCH. We hereby present two rare cases of non-LCH in pediatric patients, presented with JXG and BCH. The dermoscopic examination of both cases showed a setting-sun appearance, while the histopathological examination revealed Touton giant cells in the JXG case, and massive lymphocyte infiltration in the BCH case. Both patients were treated with 1% topical rapamycin in a split-side comparison for the first 12 weeks, followed by applications on both sides for a total duration of 24 weeks. As a result, there was a significant reduction in the size of the lesion, leading to patient's satisfaction. Rapamycin is an immunosuppressive agent with antineoplastic activity. Rapamycin can be used as an alternative non-invasive topical treatment option for JXG and BCH. However, long-term observations are required to assess its effectiveness and side effects.
PubMed: 35967913
DOI: 10.2147/CCID.S375995 -
La Clinica Terapeutica 2022Neurofibromatosis type 1 (NF1), is a rare genetic disorder that may involve almost every organ system in the body such as cutaneous, ophthalmologic and central and... (Review)
Review
Juvenile xanthogranuloma in neurofibromatosis type 1. Prevalence and possible correlation with lymphoproliferative diseases: experience of a single center and review of the literature.
Neurofibromatosis type 1 (NF1), is a rare genetic disorder that may involve almost every organ system in the body such as cutaneous, ophthalmologic and central and peripheral nervous system. Cutaneous findings are usually the first sign of the disease. In this study, we investigate the real prevalence of xanthogranulomas juvenile (JXG) and possible correlation with lymphoproliferative diseases. This is a retrospective study conducted on a population with NF1 followed by February 1983 to February 2022 at the "Sapienza" University of Rome, Italy. We investigate the real prevalence of juvenile xanthogranuloma in NF1 and possible correlation with lymphoproliferative diseases. JXG was present in 39 cases (3.1%). JXG is more frequent in NF1 than in the general population while the possible association with lymphoproliferative diseases in NF1 remains controversial.
Topics: Humans; Neurofibromatosis 1; Prevalence; Retrospective Studies; Skin; Xanthogranuloma, Juvenile
PubMed: 35857053
DOI: 10.7417/CT.2022.2445 -
Ear, Nose, & Throat Journal Jul 2022
PubMed: 35791499
DOI: 10.1177/01455613221113795 -
Archives of Disease in Childhood Sep 2022
PubMed: 35728938
DOI: 10.1136/archdischild-2022-324421 -
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 -
Indian Pediatrics Jun 2022
Topics: Glottis; Humans; Xanthogranuloma, Juvenile
PubMed: 35695144
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 -
Dermoscopy of juvenile xanthogranuloma: a retrospective descriptive study on 35 paediatric patients.Journal of the European Academy of... Oct 2022
Topics: Child; Dermoscopy; Diagnosis, Differential; Humans; Retrospective Studies; Xanthogranuloma, Juvenile
PubMed: 35686664
DOI: 10.1111/jdv.18316