Acral hemosideric lymphatic malformation.

Abstract:

BACKGROUND:Cutaneous lymphatic malformations represent a group of heterogeneous diseases caused by developmental defects of lymphatic system. OBJECTIVE:The purpose of this study was to report the clinical, histopathological and immunohistochemical features of a distinctive lymphatic malformation. METHODS: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. RESULTS: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. CONCLUSION: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.

journal_name

J Cutan Pathol

authors

Wang L,Gao T,Wang G

doi

10.1111/cup.12143

subject

Has Abstract

pub_date

2013-07-01 00:00:00

pages

657-60

issue

7

eissn

0303-6987

issn

1600-0560

journal_volume

40

pub_type

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