Histiocytoses – Xanthoma Disseminatum

March 17th, 2012

Xanthoma Disseminatum is a type of Non-X Histiocytosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices.  Clinically, Xanthoma Disseminatum appears as yellow/red/brown papules and nodules that can occur anywhere on the skin but are most prevalent in the folds and creases as well as the eyelids.  Over time, the papules and nodules become larger and can coalesce into larger plaques that encompass a significant body surface area.  Most commonly this skin disease occurs in children and young adults.  Approximately 50% of patients with Xanthoma Disseminatum have mucous membrane involvement.  The larynx and oropharynx can be involved leading to airway obstruction and difficulty breathing and talking.  The eyes can be involved as well leading to blindness.  In some instances the pituitary gland may be involved and up to 40% of patients with Xanthoma Disseminatum have diabetes insipidus.  Lipid levels are elevated in up to 20% of patients with Xanthoma Disseminatum.  Although this condition may spontaneously resolve, patients need close internal medicine surveillance and medicines such as cyclophosphamide have been successful in some patients.

Xanthoma Disseminatum