Histopathological hallmarks of cutaneous lesions of capillary malformation-arteriovenous malformation syndrome
| Autor: | Ana Martín-Santiago, José Manuel Azaña, M Valdivielso-Ramos, Ricardo Moreno, Isabel Colmenero, Almudena Hernández-Núñez, Beatriz Berenguer, Gloria Garnacho, Antonio Torrelo, Minia Campos |
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| Rok vydání: | 2019 |
| Předmět: |
Pathology
medicine.medical_specialty Capillary malformation Port-Wine Stain P120 GTPase Activating Protein Dermatology Arteriovenous Malformations 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine Dermis medicine Humans Retrospective Studies business.industry Vascular malformation Port-wine stain Endothelial Cells Nevus roseus Arteriovenous malformation p120 GTPase Activating Protein medicine.disease Capillaries Infectious Diseases medicine.anatomical_structure Spain 030220 oncology & carcinogenesis Histopathology business |
| Zdroj: | Journal of the European Academy of Dermatology and Venereology : JEADVReferences. 34(10) |
| ISSN: | 1468-3083 |
| Popis: | Importance Capillary malformation-arteriovenous malformation (CM-AVM) syndrome is a recently described syndrome with distinctive cutaneous lesions. Very little is known about the histopathology of these lesions. Objective The purpose of the study was to evaluate the histopathological characteristics of the pink macules of the CM-AVM syndrome and to investigate if these pink macules could be classified as capillary malformations or arteriovenous malformations based on their histopathological features. Design-settings-participants We conducted a retrospective multicenter study involving eight hospitals in Spain. Fifteen biopsies from pink macules of the CM-AVM syndrome were analysed and compared with five biopsies of diverse capillary malformations and three stage I arteriovenous malformations. Results Pink macules' biopsies of the CM-AVM syndrome showed similar features including a high vascular density encompassing capillaries and numerous thick-walled arterioles mainly located in the superficial dermis, a predominance of elongated over round vessels, scarce or absent erythrocytes within the lumina and discrete perivascular inflammation. CMs were characterized by an increased number of capillary-type vessels mostly rounded and located in the upper dermis. AVMs were composed by highly increased numbers of vessels with a branching pattern involving the full thickness of the dermis, without erythrocytes within the lumina. Wilms tumour 1 protein was positive in the endothelial cells both in pink macules of the CM-AVM and in arteriovenous malformations. Conclusions and relevance Pink macules of the CM-AVM syndrome seem to be different from capillary malformations. Our results suggest that histologically and immunohistochemically they are closer to incipient arteriovenous malformations than to capillary malformations. A deepened knowledge about the nature of these skin lesions will contribute to the better understanding of capillary malformation-arteriovenous malformation syndrome, and will open the possibility of new and more specific treatments in the future. |
| Databáze: | OpenAIRE |
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