
==== Front
Clin Case Rep
Clin Case Rep
10.1002/(ISSN)2050-0904
CCR3
Clinical Case Reports
2050-0904
John Wiley and Sons Inc. Hoboken

10.1002/ccr3.8912
CCR38912
CCR3-2024-02-0456.R1
Dentistry
Dermatology
Case Report
Case Report
Juvenile xanthogranuloma manifesting in the forehead: A case report
Chindia et al.
Chindia M. L. 1
Butt F. M. A. https://orcid.org/0000-0001-6353-215X
2 fawziamaxfax@gmail.com

Mung’ania M. 3
Owino R. O. 4
1 Oral & Maxillofacial Surgery University of Nairobi Nairobi Kenya
2 Human Anatomy University of Nairobi Nairobi Kenya
3 Gertrudes Children Hospital Nairobi Kenya
4 Paediatric Dentistry and Orthodontics University of Nairobi Nairobi Kenya
* Correspondence
F. M. A. Butt, Human Anatomy, University of Nairobi, Nairobi, Kenya.
Email: fawziamaxfax@gmail.com

02 9 2024
9 2024
12 9 10.1002/ccr3.v12.9 e891216 4 2024
25 2 2024
01 5 2024
© 2024 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd.
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

Key Clinical Message

A 3 ‐year‐old boy presented with a forehead nodular mass, which was excised and confirmed histologically as Juvenile Xanthogranulomma (JXG). It affects children with a predilection for the head and neck region. A relatively rare, benign, histiocytic proliferative cutaneous disorder with a potential for malignancy. A prompt and wide resection is recommended.

Juvenile Xanthogranuloma.

children
head and neck
juvenile xanthogranuloma
source-schema-version-number2.0
cover-dateSeptember 2024
details-of-publishers-convertorConverter:WILEY_ML3GV2_TO_JATSPMC version:6.4.8 mode:remove_FC converted:02.09.2024
Chindia ML , Butt FMA , Mung’ania M , Owino RO . Juvenile xanthogranuloma manifesting in the forehead: A case report. Clin Case Rep. 2024;12 :e8912. doi:10.1002/ccr3.8912
==== Body
pmc1 INTRODUCTION

Juvenile Xanthogranuloma (JXG) is a relatively uncommon, benign, histiocystic proliferative cutaneous disorder that typicaly affects children, with the head and neck being the most common sites. 1 The lesion is a non‐Langerhans cell histocytosis whose exact prevalence remains unknown. 2 Cutaneous lesions are usually asymptomatic and most of them spontaneously involute over a course of several years. 3

JXG is a proliferative disorder of histiocystic cells of the dermal dendrocyte phenotype and is diagnosed clinically and confirmed after biopsy for histological analysis. 3 We present a case of JXG prominently manifesting in the forehead of a 3‐year‐old boy.

2 CASE REPORT

2.1 Case history/examination

A 3‐year‐old boy first presented with a forehead firm, nontender mass that had evolved over about 1 month. During a follow up appointment after 1 week, ulceration of the mass (Figure 1) was noted for which his parents confirmed traumatic injury.

FIGURE 1 Clinical Photo. A traumatically ulcerated forehead nodular lesion.

3 METHODS

A haemogram indicated a full range of normal parameters with hemoglobin at 11.9 g/dL. A differential diagnosis of Langerhans cell histiocytosis, Xanthomatous lesions, dermatofibromas and the Spitz nevus was made pending the definitive one.

3.1 Treatment and Investigation

The treatment plan was excision under general anesthesia. The lesion was excised through an elliptical incision and closure accomplished utilizing a one layer monocryl suturing. The tissue was sent for histopathology evaluation. The patient has been follow up for 6 months and the recovery has been uneventful.

4 RESULTS

Histological analysis showed a delimited completely excised lesion of proliferated histiocystes admixed with lymphocytes, foamy microphages and touton type giant cells. The features were consistent with a benign histiocytic lesion the JXG (Figure 2A,B).

FIGURE 2 (A) (×4 magnification). Histological Illustration of a well dermacated lesion delimited completely excised lesion. (B) (×400 magnification) of proliferated histiocytes admixed with lymphocytes foamy macrophages and touton type giant cells.

5 DISCUSSION (TREATMENT AND FOLLOW‐UP)

JXG is a rare non‐Langerhans cell histiocytic disorder which was first described in 1905 by Adawson as a congenital xanthoma multiplex that belongs to a group of histiocytic lesions with a macrophage heterogenous phonotype and variable factor XIIIa and fascin reactivity. 4 One study outlined the diagnostic criteria for JXG, as typical Touton cells resette shaped nuclei amid eosinophilic or vacuolar cytoplasm, this was in keeping with the patients histopathology. 5 , 6

The true incidence of JXG may be underestimated since many lesions, especially those which are solitary and small (in up to 90% of the patients), may go unrecognized. 3 However, lesions manifesting in exposed areas as the present case are noted early and should raise concern for prompt intervention. Furthermore, traumatic ulceration may commonly occur during play.

Meticulous histopathological analysis should be performed in order to rule out concurrent systemic malignant processes. The etiology of JXG is unknown although some authors postulate the existence of an alteration of the macrophage response to a nonspecific stimulus such as trauma and viral infection, a hypothesis that still lacks evidence. 7 This lesion which has predilection for the eyes is rarely linked to systemic manifestations. 8 It has been recommended that enlarging soft tissue masses in children in whom a malignancy cannot be excluded by physical or radiological examination be totally excised where feasible, when no functional compromise from surgery is anticipated. 9 As for the systemic Langerhan cell histiocytosis the treatment protocol is chemotherapy and for the Central nervous system, the management may not have an effect on the patients mortality. 10 While in the present case aesthetic compromise was notable, the incision design was intended to extirpate the entire lesion. A month after follow up of the surgical intervention the linear scar had healed well with no evidence of hypertrophy. The patient remains under review for over at least 6 months.

AUTHOR CONTRIBUTIONS

Fawzia Butt: Data curation; writing – review and editing. M. L. Chindia: Conceptualization; supervision. M. Mung’ania: Data curation. Richard Owino: Methodology.

FUNDING INFORMATION

There was no funding available for this report.

CONFLICT OF INTEREST STATEMENT

None.

DATA AVAILABILTY STATEMENT

Data supporting this case report will be available once this article has a accession number.

CONSENT

Written informed consent was obtained from the patient to publish this report in accordance with the journal’s patient consent policy.

ACKNOWLEDGMENTS

We greatly appreciate Claris Kamula for skillfully preparing the manuscript.
==== Refs
REFERENCES

1 Saifaldein AA , Almahmoud FH , Babgi RI , Alsammahi AA . A subcutaneous juvenile Xanthogranuloma in a 4‐ year‐old girl who presented with a lower eyelid mass. Ophthalmol. 2019;10 (2 ):153‐159.
2 Saiful JH , Gustia R . A rare case of juvenile Xanthogranuloma in an 8‐month ‐old baby with Dyslipemia.Journal of biomedicine. Transl Res. 2021;5 :565‐566.
3 Collie JS , Harper CD , Fillma EP . Juvenile Xanthogramiloma. 2023. https://www.ncbi.nlm.nih.gov/books/NBK 526103/
4 Yuso Y , Muhammad J , Ahmad I , Kew CH , Teoh PY , Hamzah NH . Juvenile xanthomagranuloma: A case report and literature review. J Gene Med. 2022;19 (4 ):380. https://dol.org/10.293‐33/ejgm/12029
5 Haroche J , Abla O . Uncommon histiocytic disorders: Rosai‐Dorfman, juvenile xanthogranuloma, and Erdheim‐Chester disease. Hematology Am Soc Hematol Educ Program. 2015;2015 :571‐578. doi:10.1182/asheducation-2015.1.571 PMID: 26637774.26637774
6 Haupt R , Minkov M , Astigarraga I , et al. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work‐up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer. 2013;60 (2 ):175‐184. doi:10.1002/pbc.24367 Epub 2012 Oct 25. PMID: 23109216; PMCID: PMC4557042.23109216
7 Hermandez‐san MMJ , Vargas‐Mora P , Aranibar L . Juvenile Xanthogranuloma: an Entity with a wide clinical spectrum. Sch J Med Case Rep. 2020;111 :725‐733.
8 Lim LT , Mclaghlin S , Lavy T , Penman D , Dutton GN . Juvenile Xanthogranuloma: an unusual eyelid presentation. Eye. 2010;24 :1425‐1426.20186166
9 Cypel TKS , Zuker RM , Juvenile Xanthogranuloma: Case report and review of the literature, (2000); 16 :175–177.
10 Lian H , Wei A , He L , et al. Clinical analysis of pediatric systemic juvenile Xanthogranulomas: a retrospective single‐center study. Front Pediatr. 2021;9 :672547. doi:10.3389/fped.2021.672547 34178890
