Xanthoma disseminatum: Effective therapy with 2-chlorodeoxyadenosine in a case series

Farzaneh Khezri, Lawrence E. Gibson, Ayalew Tefferi

Research output: Contribution to journalArticle

28 Citations (Scopus)

Abstract

Background: Xanthoma disseminatum is a rare non-familial 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. Observations: 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. Conclusion: Among 8 cases of xanthoma disseminatum, a positive response to treatment with 2-chlorodeoxyadenosine was seen in 5 cases.

Original languageEnglish (US)
Pages (from-to)459-464
Number of pages6
JournalArchives of Dermatology
Volume147
Issue number4
DOIs
StatePublished - Apr 2011

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Non-Langerhans-Cell Histiocytosis
Cladribine
Therapeutics
Skin
Chronic Disease
Cell Proliferation
Lipids

ASJC Scopus subject areas

  • Dermatology

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Xanthoma disseminatum : Effective therapy with 2-chlorodeoxyadenosine in a case series. / Khezri, Farzaneh; Gibson, Lawrence E.; Tefferi, Ayalew.

In: Archives of Dermatology, Vol. 147, No. 4, 04.2011, p. 459-464.

Research output: Contribution to journalArticle

Khezri, Farzaneh ; Gibson, Lawrence E. ; Tefferi, Ayalew. / Xanthoma disseminatum : Effective therapy with 2-chlorodeoxyadenosine in a case series. In: Archives of Dermatology. 2011 ; Vol. 147, No. 4. pp. 459-464.
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