Adult onset xanthogranuloma – case report and review of literature

Juvenile xanthogranuloma represents the most common form of non-Langerhans cell histiocytosis. It is tipically a childhood disorder, over 80% of cases developing in the first year of life. Adult onset xanthogranuloma is instead exceedingly rare; our literature research revealed a total number of only 35 cases reported to date. Cases with multiple cutaneous lesions occurring during adulthood are even rarer. The pathogenesis of the disease is incompletely understood and the possibility of extracutaneous involvement is uncertain.Although adult xanthogranulomatosis is not considered a paraneoplastic phenomenon, association with haematological malignancies is very frequent, therefore patients should be thoroughly investigated and closely monitored. We report a case of multiple xanthogranulomas developing in an otherwise healthy adult female patient and review the literature regarding this uncommon disease.