Klin Monbl Augenheilkd 2011; 228(4): 379-380
DOI: 10.1055/s-0031-1273238
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© Georg Thieme Verlag KG Stuttgart · New York

Retinal Manifestations in a Patient with Xanthoma Disseminatum (Non-Langerhans Cell Histiocytosis)

Retinale Manifestation bei einem Patienten mit Xanthoma disseminatum (Non-Langerhanszell-Histiozytose)L. Konstantinidis, E. Laffitte, Y.-M. Wasem, K. Balaskas, Y. Guex-Crosier
Further Information

Publication History





Publication Date:
11 April 2011 (online)

Background

Xanthoma disseminatum (XD) is a rare normolipemic histiocytic disorder of non-Langerhans cell origin. It is a chronic systemic disease with a benign course, characterized by disseminated xanthomatous lesions. It typically involves the skin, mainly the flexor skin folds and eyelids.[1] XD may also manifest in the central nervous system, respiratory system and gastrointestinal tracts [1] [2].

Since this condition was first described in 1938 by Montgomery and Osterberg [3], few cases have been reported in the literature. Furthermore, apart from the eyelids, only sporadic reports of ocular involvement have been described with episcleral and corneal manifestations [4] [5]. As far as we are aware this is the first report of retinal manifestations related to this rare clinical entity.

References

  • 1 Zak I T, Altinok D, Neilsen S S et al. Xanthoma disseminatum of the central nervous system and cranium.  Am J Neuroradiol. 2006;  27 919-921
  • 2 Alexander A S, Turner R, Uniate L et al. Xanthoma disseminatum: a case report and literature review.  Br J Radiol. 2005;  78 153-157
  • 3 Montgomery H, Osterberg A E. Xanthomatosis: correlation of clinical, histopathological and chemical studies of cutaneous xanthoma.  Arch Dermatol Syphilol. 1938;  37 373-402
  • 4 Stirling R J, Bradbury J. Corneal involvement in xanthoma disseminatum.  Eye. 1998;  12 317-318
  • 5 Wayman L L, Margo C E. Xanthoma disseminatum with bilateral epibulbar involvement.  Am J Ophthalmol. 2005;  139 557-559 

Yan Guex-Crosier, MD, FEBO

Head of uveitis clinic, Jules Gonin Eye Hospital, University of Lausanne

15 Av. de France

1004 Lausanne

Switzerland

Phone: ++ 41/21/6 26 85 95

Fax: ++ 41/21/6 26 81 22

Email: yan.guex@fa2.ch