Background: Papular xanthoma (PX) is a rare normolipidemic non-Langerhans cell histiocytoxanthomatosis affecting both children and adults. Objective: We describe an adult case of PX associated with angiokeratoma of Fordyce and review the literature in order to compare and discuss previous reports. Methods: We studied the clinical, histopathological, immunocytochemical and ultrastructural findings. Results: We report the findings of our case and compare our case with those described in the literature. Conclusions: Three clinical patterns of PX appeared to emerge in the review of the literature: a self-healing form, a persistent form and a progressive form. The progressive form of PX can be considered the same clinical entity that is also described as progressive nodular histiocytosis.

Keywords:
Papular xanthoma, Non-Langerhans cell histiocytoxanthomatosis, Progressive nodular histiocytosis
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2003
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