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- Title
CR40 Exceptionally large juvenile xanthogranuloma – a case report.
- Authors
Bulić, Luka; Brenner, Eva; Bulić, Suzana Ožanić
- Abstract
INTRODUCTION/OBJECTIVES: Juvenile xanthogranuloma (JXG) is a rare, benign skin lesion pathologically classified as a non-Langerhans cell histiocytosis. The lesions appear within the first year of life in 75% of patients, predominantly on the head or neck, growing up to 5mm in size. While the etiology is mostly infectious, it can also be caused by genetic variants. In most patients, the condition has an easy course and resolution. Histopathological features include a histiocytic invasion of the superficial dermis, with additional eosinophils, lymphocytes and plasma cells. The lesion typically stains with anti-CD4, anti-XIIIa and CD68 markers. CASE PRESENTATION: A 1-year-old female patient was examined for a large, solitary scalp lesion with features of JXG. The lesion started as a yellow erythematous plaque, measuring 17x13mm in size. Histopathological analysis showed histiocytes and occasional foam cells, lymphocytes, macrophages and multinuclear giant cells in the dermis. Immunodiagnostic tests returned positive for CD68 and negative for S100 and CD1a, confirming JXG. The patient attended three follow-up appointments, at which substantial growth of the lesion was observed. At the last examination, it measured 40mm in diameter. Topical corticosteroids were applied but didn’t affect the progression. CONCLUSION: This case of JXG is set apart by the size of the lesion, which is eight times larger than in the average patient. Since the patient had no prior infection or serious illness, it is worth raising the question of a genetic variant being the cause of the lesion, and how such a variant might have impacted its size.
- Subjects
JUVENILE xanthogranuloma; NON-langerhans-cell histiocytosis; FOAM cells; PLASMA cells; GENETIC variation; ORGAN transplant waiting lists
- Publication
Lijecnicki Vjesnik, 2023, Vol 145, p66
- ISSN
0024-3477
- Publication type
Article
- DOI
10.26800/LV-145-supl2-CR40