Port-wine stains. Morphologic variations and developmental lesions

Abstract

Studies have shown that port-wine stains (PWSs) probably represent a progressive ectasia of the superficial cutaneous vascular plexus. During examination of approximately 500 patients with PWSs, seven lesions were identified that could be categorized into two groups. In one group (two patients), the typical superficial PWS was accompanied by a proliferation and ectasia of thin-walled vessels in the deeper dermis, subcutaneous adipose tissue, and skeletal muscle (cavernous hemangioma). In the other group (five patients), single or multiple polypoid tumors arose that consisted of a proliferation of vessels with walls of varying thickness, and intervascular stroma (arteriovenous malformation). These tumors appear to arise in both young patients with flat lesions as well as the older patients with pronounced surface irregularities. Two patients were specifically chosen because they represented the typical PWS cobblestone pattern. This vascular abnormality represents a localized exaggeration of the ectasia process. We conclude that a small number of PWSs are associated with an underlying cavernous hemangioma. Furthermore, besides the surface irregularities that characterize the older PWS, de novo vascular tumors arise that can be categorized as arteriovenous malformations.

Progressive Macular Hemangioma: A New Entity?

“…A typical PWS on the other hand is classified as an ectasia of the cutaneous vascular plexus that is almost always present at birth [1]. Since there is no element of angiogenesis, the area of the lesion relative to the body surface area remains the same, ‘as a rule’ [1].…”

“…Since there is no element of angiogenesis, the area of the lesion relative to the body surface area remains the same, ‘as a rule’ [1]. The only visible change is a darkening in color from light pink to a deeper red-purple and a progressive ‘cobblestoning’ of the surface, which is seen most often over facial lesions.…”

“…Even when other body sites are involved, there is a sharp midline cutoff. Their size remains constant relative to the body surface area, with thickening and cobblestoning seen over lesions with the passage of time [1]. …”

Morphological characteristic of capillary malformations of head and neck

“…Их наличие позволяет предположить, что капиллярные мальформации со временем могут преобразовываться в капиллярно-артериовенозную форму и, возможно, в артериовенозную форму, что совпадает с данными J.L. Finley et al (1984). Не исключено, что узелковую форму капиллярных мальформаций можно рассматри-вать как развитие третьего вида простой мальформации и как начальную стадию формирования артериовенозной мальформации или смешанной формы.…”

A Clinical Study of Hemangiomas and Vascular Malformations

“…It may progressively darken over many years, and occasional lesions develop secondary proliferative vascular blebs on their surface(cobble stone appearance). [7] PDL therapy is usually performed over several sessions, separated in time by 6-8 weeks, and can be quite effective in lightening these lesions. [8] …”