Acral hemosideric lymphatic malformation
Article first published online: 23 MAR 2013
© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Journal of Cutaneous Pathology
Volume 40, Issue 7, pages 657–660, July 2013
How to Cite
Acral hemosideric lymphatic malformation., , .
- Issue published online: 16 JUN 2013
- Article first published online: 23 MAR 2013
- Accepted manuscript online: 6 MAR 2013 03:34PM EST
- Manuscript Accepted: 2 MAR 2013
- Manuscript Revised: 27 FEB 2013
- Manuscript Received: 6 FEB 2013
- vascular malformation;
Cutaneous lymphatic malformations represent a group of heterogeneous diseases caused by developmental defects of lymphatic system.
The purpose of this study was to report the clinical, histopathological and immunohistochemical features of a distinctive lymphatic malformation.
Twelve patients with similar clinical and histopathological features were included in this study. Immunohistochemical staining of CD31, D2-40, Prox1 and Wilms tumor 1 (WT-1) were performed on all lesions.
All cases were either congenital lesions or developed during the first 2 years of life. All presented as red to brown papules or nodules on acral sites. Histopathologically, the lesions consisted of a dermal proliferation of flat or slit-like vessels lined with a single layer of endothelial cells. Hemosiderin or extravascular red blood cells were present in all cases. The constituent vessels expressed CD31, D2-40 and Prox1 and lacked expression of WT-1.
On the basis of the clinical, histopathological and immunohistochemical findings, our cases represent a unique type of lymphatic malformation that we believe is distinct from previously reported vascular proliferations. We propose the name of acral hemosideric lymphatic malformation for this entity.