Characterization of intravascular papillary endothelial hyperplasia: a multicentre cohort

Intravascular papillary endothelial hyperplasia (IPEH) is a rare, benign vascular lesion typically presenting in the skin as a subcutaneous nodule, which may be clinically mistaken for other neoplasms such as haemangiomas and lipomas. We found that most clinicians' initial impressions prior to biopsy did not include the final diagnosis of IPEH, often using vague terms such as ‘soft tissue mass’, which indicates a potential need for greater awareness of this condition. Given that the differential diagnosis of IPEH often includes conditions such as melanoma or angiosarcoma, clinicopathological correlation is of utmost importance for this uncommon vascular lesion.

Summary

Intravascular papillary endothelial hyperplasia (IPEH) is an uncommon benign malformation of the skin and subcutaneous tissue. In this retrospective multicentre study, we aimed to investigate the clinical and pathological features of 261 patients with IPEH. IPEH is classified into three categories; in our study, the proportions were pure (50%), mixed (46%) and extravascular (4%). IPEH frequently stained positive for immunohistochemical markers such as CD31, CD34, smooth muscle actin and erythroblast transformation-specific-related gene. Clinicians' initial impression of the lesion often included ambiguous terms such as ‘soft tissue mass’. There is an opportunity for increased awareness of this lesion and its consideration within a differential diagnosis.