Papular xanthoma associated with angiokeratoma of Fordyce: considerations on the nosography of this rare non-Langerhans cell histiocytoxanthomatosis.
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.
Caputo
Ruggero
R
Institute of Dermatological Sciences, University of Milan, IRCCS Ospedale Maggiore of Milan, Italy. ruggero.caputo@unimi.it
Passoni
Emanuela
E
Cavicchini
Stefano
S
D000328:Adult; D000794:Angiokeratoma / Q000150:complications* / Q000473:pathology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications* / Q000473:pathology; D006801:Humans; D008297:Male; D010413:Penis; D012611:Scrotum; D012878:Skin Neoplasms / Q000150:complications* / Q000473:pathology; D014973:Xanthomatosis / Q000150:complications* / Q000473:pathology