Cargando…

Familial Disseminated Cutaneous Glomuvenous Malformation: Treatment with Polidocanol Sclerotherapy

Glomuvenous malformations (GVMs) present as asymptomatic multiple pink-to-blue nodules or plaques. Disseminated lesions are rare, representing 10% of all the cases. Familial cases are caused by mutations in the glomulin gene. A young male presented with multiple bluish-to-dusky red-coloured nodules...

Descripción completa

Detalles Bibliográficos
Autores principales: Jha, Aditi, Khunger, Niti, Malarvizhi, K, Ramesh, V, Singh, Avninder
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5227083/
https://www.ncbi.nlm.nih.gov/pubmed/28163461
http://dx.doi.org/10.4103/0974-2077.197083
_version_ 1782493757733601280
author Jha, Aditi
Khunger, Niti
Malarvizhi, K
Ramesh, V
Singh, Avninder
author_facet Jha, Aditi
Khunger, Niti
Malarvizhi, K
Ramesh, V
Singh, Avninder
author_sort Jha, Aditi
collection PubMed
description Glomuvenous malformations (GVMs) present as asymptomatic multiple pink-to-blue nodules or plaques. Disseminated lesions are rare, representing 10% of all the cases. Familial cases are caused by mutations in the glomulin gene. A young male presented with multiple bluish-to-dusky red-coloured nodules 10–15 in numbers over the trunk, limbs and buttocks since 12 years of age. They ranged in size from 1 to 3 cm, partially to non-compressible and tender on palpation. There was no history of any systemic complaint. His sister and mother had similar lesions but in a limited distribution. Biopsy showed multiple ectatic dilated vascular channels lined by multiple layers of glomus cells consistent with the diagnosis of GVM. The biopsy of the lesions from the mother and sister also showed similar features. Mutation analysis for glomulin gene could not be done because of the unavailability of the facility at our setting. He underwent sclerotherapy with 3% polidocanol every 2 weeks, and there was significant improvement in the lesions after six sessions of sclerotherapy. The patient is under follow-up and there is no recurrence of the lesions over treated sites after 6 months.
format Online
Article
Text
id pubmed-5227083
institution National Center for Biotechnology Information
language English
publishDate 2016
publisher Medknow Publications & Media Pvt Ltd
record_format MEDLINE/PubMed
spelling pubmed-52270832017-02-03 Familial Disseminated Cutaneous Glomuvenous Malformation: Treatment with Polidocanol Sclerotherapy Jha, Aditi Khunger, Niti Malarvizhi, K Ramesh, V Singh, Avninder J Cutan Aesthet Surg Case Report Glomuvenous malformations (GVMs) present as asymptomatic multiple pink-to-blue nodules or plaques. Disseminated lesions are rare, representing 10% of all the cases. Familial cases are caused by mutations in the glomulin gene. A young male presented with multiple bluish-to-dusky red-coloured nodules 10–15 in numbers over the trunk, limbs and buttocks since 12 years of age. They ranged in size from 1 to 3 cm, partially to non-compressible and tender on palpation. There was no history of any systemic complaint. His sister and mother had similar lesions but in a limited distribution. Biopsy showed multiple ectatic dilated vascular channels lined by multiple layers of glomus cells consistent with the diagnosis of GVM. The biopsy of the lesions from the mother and sister also showed similar features. Mutation analysis for glomulin gene could not be done because of the unavailability of the facility at our setting. He underwent sclerotherapy with 3% polidocanol every 2 weeks, and there was significant improvement in the lesions after six sessions of sclerotherapy. The patient is under follow-up and there is no recurrence of the lesions over treated sites after 6 months. Medknow Publications & Media Pvt Ltd 2016 /pmc/articles/PMC5227083/ /pubmed/28163461 http://dx.doi.org/10.4103/0974-2077.197083 Text en Copyright: © 2016 Journal of Cutaneous and Aesthetic Surgery http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle Case Report
Jha, Aditi
Khunger, Niti
Malarvizhi, K
Ramesh, V
Singh, Avninder
Familial Disseminated Cutaneous Glomuvenous Malformation: Treatment with Polidocanol Sclerotherapy
title Familial Disseminated Cutaneous Glomuvenous Malformation: Treatment with Polidocanol Sclerotherapy
title_full Familial Disseminated Cutaneous Glomuvenous Malformation: Treatment with Polidocanol Sclerotherapy
title_fullStr Familial Disseminated Cutaneous Glomuvenous Malformation: Treatment with Polidocanol Sclerotherapy
title_full_unstemmed Familial Disseminated Cutaneous Glomuvenous Malformation: Treatment with Polidocanol Sclerotherapy
title_short Familial Disseminated Cutaneous Glomuvenous Malformation: Treatment with Polidocanol Sclerotherapy
title_sort familial disseminated cutaneous glomuvenous malformation: treatment with polidocanol sclerotherapy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5227083/
https://www.ncbi.nlm.nih.gov/pubmed/28163461
http://dx.doi.org/10.4103/0974-2077.197083
work_keys_str_mv AT jhaaditi familialdisseminatedcutaneousglomuvenousmalformationtreatmentwithpolidocanolsclerotherapy
AT khungerniti familialdisseminatedcutaneousglomuvenousmalformationtreatmentwithpolidocanolsclerotherapy
AT malarvizhik familialdisseminatedcutaneousglomuvenousmalformationtreatmentwithpolidocanolsclerotherapy
AT rameshv familialdisseminatedcutaneousglomuvenousmalformationtreatmentwithpolidocanolsclerotherapy
AT singhavninder familialdisseminatedcutaneousglomuvenousmalformationtreatmentwithpolidocanolsclerotherapy