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Keywords:

  • D2-40;
  • digit;
  • lymphatic;
  • malformation;
  • Prox1;
  • vascular proliferation;
  • WT-1

Background

Cutaneous lymphatic malformations represent a group of heterogeneous diseases. In clinical practice, some cases of cutaneous lymphatic malformation have been found to differ from the well-characterized entities, and are difficult to classify.

Objective

To report the clinical, histopathological and immunohistochemical characteristics of six cases of lymphatic malformation characterized by swelling of the digits.

Methods

Six patients with swelling of the digits were included in this study. Histopathological examination and immunohistochemical staining of CD31, D2-40, Prox1, Wilms tumor 1 (WT-1) and human herpesvirus-8 (HHV-8) were performed for all cases.

Results

All cases were congenital lesions and presented as swelling of single or multiple digits. Histopathologically, the lesions showed proliferation of small-to-medium-sized vessels in the dermis and subcutaneous tissue. These abnormal vessels were positive for CD31, D2-40 and Prox1, and negative for WT-1 and HHV-8.

Conclusion

The six cases reported herein represent a specific cutaneous lymphatic malformation different from previously reported entities.