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Keywords:

  • D2-40;
  • hemosiderin;
  • Prox1;
  • vascular malformation;
  • WT-1

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.