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Journal of the American Academy of Dermatology
Volume 50, n° 4
pages 608-612 (avril 2004)
Doi : 10.1016/j.jaad.2003.04.002
Epithelial and mesenchymal hamartomatous changes in a mature port-wine stain: morphologic evidence for a multiple germ layer field defect
 

Ignacio Sanchez-Carpintero, MD, PhD a, Martin C Mihm, MD a, b, Adam Mizeracki, BS b, c, Milton Waner, MD b, Paula E North, MD, PhD b, c,
a Department of Pathology, Massachusetts General Hospital and Harvard University, Boston, Massachusetts, USA 
b Department of Otolaryngology, Arkansas Children's Hospital and University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA 
c Department of Pathology, Arkansas Children's Hospital and University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA 

*Reprint requests: Paula E. North, MD, PhD, Arkansas Children's Hospital, 800 Marshall St, Little Rock, AR 72202, USA.
Abstract

The port-wine stain (PWS) is a congenital cutaneous venulocapillary malformation of unknown pathogenesis. Many patients with facial PWS develop thickening with cobblestoning and nodularity during adult life. The histologic correlates of this maturational change are poorly documented and its mechanisms remain unclear. In this case study we present new histologic observations that may elucidate this phenomenon. An extensive PWS on the face of a 75-year-old man exhibited gross thickening with cobblestoning and nodularity. Histologic examination revealed not only the expected vascular abnormalities, but also a number of widely distributed epithelial, neural, and mesenchymal hamartomatous changes. Epithelial changes included epidermal nevus, sebaceous trichofolliculoma, and basaloid follicular hamartoma. Changes of connective tissue nevus, smooth-muscle hamartoma, neural hamartoma, and subcuticular hamartoma were also noted. The complex hamartomatous changes observed in the PWS of this patient involved multiple germ lines and were distributed in a widespread pattern. These changes not only offer an explanation for the skin thickening and nodularity of this patient, but also suggest a genetically determined, multilineage developmental field defect in the pathogenesis of this lesion. Further studies of other patients are necessary to understand the full implications of these findings in the late stage of PWS.

The full text of this article is available in PDF format.

 Supported by a grant from the Arkansas Children's Hospital Foundation and by funds from the Departments of Pathology and Otolaryngology at the University of Arkansas for Medical Sciences, Little Rock, Ark.
Conflicts of interest: None identified.



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