Congenital Juvenile Xanthogranuloma in the Perioral Region: A Case Image

Juvenile xanthogranuloma (JXG) is the most common form of non-Langerhans cell histiocytosis in childhood. It often presents with cutaneous involvement and exhibits a predilection for the head and neck region. This article illustrates a case of congenital JXG in a 5-month-old boy, characterized by a...

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Bibliographic Details
Published in:Head & neck pathology (Totowa, N.J.) N.J.), 2024-04, Vol.18 (1), p.35, Article 35
Main Authors: Maldonado, Andrea, Muñoz, Rubén, Alarcon, Nayari, Ventura, José Victor Lemos, de Andrade, Bruno Augusto Benevenuto, Villarroel-Dorrego, Mariana, de Arruda, José Alcides Almeida
Format: Article
Language:English
Subjects:
Dentistry
Humans
Infant
Male
Medicine
Medicine & Public Health
Oral and Maxillofacial Surgery
Otorhinolaryngology
Pathology
Xanthogranuloma, Juvenile - congenital
Xanthogranuloma, Juvenile - pathology
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Summary:Juvenile xanthogranuloma (JXG) is the most common form of non-Langerhans cell histiocytosis in childhood. It often presents with cutaneous involvement and exhibits a predilection for the head and neck region. This article illustrates a case of congenital JXG in a 5-month-old boy, characterized by a solitary, well-circumscribed nodule above the left upper lip. Histopathologically, the lesion exhibited histiocytes with eosinophilic cytoplasm and Touton giant cells. Immunohistochemistry revealed histiocytes positive for CD68 and Factor XIIIa, while negative for S-100 protein. Clinicians should become familiar with the broad clinical spectrum of cutaneous JXG, particularly its congenital presentation, in order to ensure timely and accurate management.
ISSN:1936-0568
1936-0568
DOI:10.1007/s12105-024-01649-y