Symmetrical Facial Giant Plaque-Type Juvenile Xanthogranuloma: Case Report and Review of the Literature
Juvenile xanthogranuloma (JXG) is the most common type of non-Langerhans cell histiocytosis. JXG is a rare benign tumor, which may be present at birth or develop later. The classical form of JXG is characterized by a red-yellowish benign papule or nodule with predilection sites on the head, neck, an...
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| Format: | Article |
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Karger Publishers
2021-07-01
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| Series: | Case Reports in Dermatology |
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| Online Access: | https://www.karger.com/Article/FullText/515151 |
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| author | Kaspar Itin Peter Häusermann Peter Itin Nicole Fosse |
| author_facet | Kaspar Itin Peter Häusermann Peter Itin Nicole Fosse |
| author_sort | Kaspar Itin |
| collection | DOAJ |
| description | Juvenile xanthogranuloma (JXG) is the most common type of non-Langerhans cell histiocytosis. JXG is a rare benign tumor, which may be present at birth or develop later. The classical form of JXG is characterized by a red-yellowish benign papule or nodule with predilection sites on the head, neck, and trunk, although lesions can appear on extremities or extracutaneous sites. In most cases there is only one lesion, whereas numerous papules or nodules may occur. Special forms of JXG such as mixed, giant, subcutaneous, eruptive, clustered, and plaque-like have been reported and associations between JXG and systemic diseases have been made. Diagnosis mainly relies on the clinical appearance, and histology usually can confirm the disease. Here we present a very rare case of symmetrical giant facial plaque-type juvenile xanthogranuloma (SGFP-JXG) and compare it with classical JXG, variations of JXG, and discuss the differential diagnosis. A 4-year-old Caucasian female presented with plaque-like lesions composed of yellowish confluent papules on both the cheeks. The histological evaluation revealed a histiocytic lesion with a formation of Touton giant cells and immunohistochemistry results confirmed the diagnosis of the SGFP-JXG. In comparison to classical JXG, the onset of SGFP-JXG sometimes occurs later and the spontaneous resolution period may be prolonged. No associated diseases and no systemic involvements were observed. Histopathology is required to differentiate this form of JXG from other histiocytosis. To the best of our knowledge, only four cases of SGFP-JXG have been reported in the literature so far. |
| first_indexed | 2024-12-22T08:29:42Z |
| format | Article |
| id | doaj.art-4635241875db4592b72a85fdf15acd66 |
| institution | Directory Open Access Journal |
| issn | 1662-6567 |
| language | English |
| last_indexed | 2024-12-22T08:29:42Z |
| publishDate | 2021-07-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Dermatology |
| spelling | doaj.art-4635241875db4592b72a85fdf15acd662022-12-21T18:32:31ZengKarger PublishersCase Reports in Dermatology1662-65672021-07-0113239940610.1159/000515151515151Symmetrical Facial Giant Plaque-Type Juvenile Xanthogranuloma: Case Report and Review of the LiteratureKaspar ItinPeter HäusermannPeter ItinNicole FosseJuvenile xanthogranuloma (JXG) is the most common type of non-Langerhans cell histiocytosis. JXG is a rare benign tumor, which may be present at birth or develop later. The classical form of JXG is characterized by a red-yellowish benign papule or nodule with predilection sites on the head, neck, and trunk, although lesions can appear on extremities or extracutaneous sites. In most cases there is only one lesion, whereas numerous papules or nodules may occur. Special forms of JXG such as mixed, giant, subcutaneous, eruptive, clustered, and plaque-like have been reported and associations between JXG and systemic diseases have been made. Diagnosis mainly relies on the clinical appearance, and histology usually can confirm the disease. Here we present a very rare case of symmetrical giant facial plaque-type juvenile xanthogranuloma (SGFP-JXG) and compare it with classical JXG, variations of JXG, and discuss the differential diagnosis. A 4-year-old Caucasian female presented with plaque-like lesions composed of yellowish confluent papules on both the cheeks. The histological evaluation revealed a histiocytic lesion with a formation of Touton giant cells and immunohistochemistry results confirmed the diagnosis of the SGFP-JXG. In comparison to classical JXG, the onset of SGFP-JXG sometimes occurs later and the spontaneous resolution period may be prolonged. No associated diseases and no systemic involvements were observed. Histopathology is required to differentiate this form of JXG from other histiocytosis. To the best of our knowledge, only four cases of SGFP-JXG have been reported in the literature so far.https://www.karger.com/Article/FullText/515151juvenile xanthogranulomasymmetrical facial plaque-type jxgnon-langerhans histiocytosistouton giant cellscd68 |
| spellingShingle | Kaspar Itin Peter Häusermann Peter Itin Nicole Fosse Symmetrical Facial Giant Plaque-Type Juvenile Xanthogranuloma: Case Report and Review of the Literature Case Reports in Dermatology juvenile xanthogranuloma symmetrical facial plaque-type jxg non-langerhans histiocytosis touton giant cells cd68 |
| title | Symmetrical Facial Giant Plaque-Type Juvenile Xanthogranuloma: Case Report and Review of the Literature |
| title_full | Symmetrical Facial Giant Plaque-Type Juvenile Xanthogranuloma: Case Report and Review of the Literature |
| title_fullStr | Symmetrical Facial Giant Plaque-Type Juvenile Xanthogranuloma: Case Report and Review of the Literature |
| title_full_unstemmed | Symmetrical Facial Giant Plaque-Type Juvenile Xanthogranuloma: Case Report and Review of the Literature |
| title_short | Symmetrical Facial Giant Plaque-Type Juvenile Xanthogranuloma: Case Report and Review of the Literature |
| title_sort | symmetrical facial giant plaque type juvenile xanthogranuloma case report and review of the literature |
| topic | juvenile xanthogranuloma symmetrical facial plaque-type jxg non-langerhans histiocytosis touton giant cells cd68 |
| url | https://www.karger.com/Article/FullText/515151 |
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