title: Xanthoma disseminatum: effective therapy with 2-chlorodeoxyadenosine in a case series.
abstract: BACKGROUND
  Xanthoma disseminatum is a rare nonfamilial disease characterized by lipid deposition in skin and internal organs due to histiocytic cell proliferation, classified as a benign non-Langerhans cell histiocytosis. This chronic disease has no known established treatment. We report 8 cases of xanthoma disseminatum with treatment outcomes in long-term follow-up and provide a review of the medical literature.
  
  
  METHODS
  We studied clinical manifestations; disease course; histopathologic and immunopathological findings; and responses to treatment in 8 consecutively seen patients with xanthoma disseminatum, including follow-up. The follow-up period ranged from 3 months to 8 years. Five patients received 2-chlorodeoxyadenosine. Therapy with 2-chlorodeoxyadenosine was effective at inducing remission and long-term control of cutaneous lesions of xanthoma disseminatum in 5 cases. Adverse effects were minimal, and treatment was well tolerated. No improvement was noted in untreated patients.
  
  
  CONCLUSIONS
  Among 8 cases of xanthoma disseminatum, a positive response to treatment with 2-chlorodeoxyadenosine was seen in 5 cases.
authors.affiliation: Department of Dermatology, Mayo Clinic, Rochester, MN 55905, USA.
authors.forename: Farzaneh
authors.initials: F
authors.lastname: Khezri
authors.forename: Lawrence E
authors.initials: LE
authors.lastname: Gibson
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
mesh_terms: D000328:Adult; D000368:Aged; D002908:Chronic Disease; D017338:Cladribine / Q000627:therapeutic use*; D005260:Female; D006644:Histiocytes / Q000187:drug effects / Q000473:pathology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D017338:Cladribine