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Encephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report

Haberland syndrome or Fishman syndrome also known as encephalocraniocutaneous lipomatosis (ECCL) is a rare, congenital neurocutaneous disorder. It is characterized by unilateral involvement of skin, eyes and central nervous system. We report the case of a 28-year-old woman who presented with soft li...

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Autores principales: Jagati, Ashish, Shah, Bela J., Joshi, Rima, Gajjar, Trusha
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/PMC5134170/
https://www.ncbi.nlm.nih.gov/pubmed/27990391
http://dx.doi.org/10.4103/2229-5178.193901
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author Jagati, Ashish
Shah, Bela J.
Joshi, Rima
Gajjar, Trusha
author_facet Jagati, Ashish
Shah, Bela J.
Joshi, Rima
Gajjar, Trusha
author_sort Jagati, Ashish
collection PubMed
description Haberland syndrome or Fishman syndrome also known as encephalocraniocutaneous lipomatosis (ECCL) is a rare, congenital neurocutaneous disorder. It is characterized by unilateral involvement of skin, eyes and central nervous system. We report the case of a 28-year-old woman who presented with soft lipomatous swelling over right temporal area with nonscarring alopecia of part of frontal and parietal region. The patient had a history of seizures and ipsilateral scleral dermoid. Computed tomography scan findings were suggestive of lipomas and calcification of falx. Magnetic resonance imaging showed right-sided hemi atrophy and two intracranial cysts. We report this case because of its rarity.
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spelling pubmed-51341702016-12-16 Encephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report Jagati, Ashish Shah, Bela J. Joshi, Rima Gajjar, Trusha Indian Dermatol Online J Case Report Haberland syndrome or Fishman syndrome also known as encephalocraniocutaneous lipomatosis (ECCL) is a rare, congenital neurocutaneous disorder. It is characterized by unilateral involvement of skin, eyes and central nervous system. We report the case of a 28-year-old woman who presented with soft lipomatous swelling over right temporal area with nonscarring alopecia of part of frontal and parietal region. The patient had a history of seizures and ipsilateral scleral dermoid. Computed tomography scan findings were suggestive of lipomas and calcification of falx. Magnetic resonance imaging showed right-sided hemi atrophy and two intracranial cysts. We report this case because of its rarity. Medknow Publications & Media Pvt Ltd 2016 /pmc/articles/PMC5134170/ /pubmed/27990391 http://dx.doi.org/10.4103/2229-5178.193901 Text en Copyright: © Indian Dermatology Online Journal 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
Jagati, Ashish
Shah, Bela J.
Joshi, Rima
Gajjar, Trusha
Encephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report
title Encephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report
title_full Encephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report
title_fullStr Encephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report
title_full_unstemmed Encephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report
title_short Encephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report
title_sort encephalocraniocutaneous lipomatosis (haberland syndrome): a rare case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5134170/
https://www.ncbi.nlm.nih.gov/pubmed/27990391
http://dx.doi.org/10.4103/2229-5178.193901
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