Open AccessEncephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report
Author(s) -
Ashish Jagati,
Bela Shah,
Rima Joshi,
Trusha Gajjar
Publication year - 2016
Publication title -
indian dermatology online journal
Language(s) - English
Resource type - Journals
eISSN - 2249-5673
pISSN - 2229-5178
DOI - 10.4103/2229-5178.193901
Subject(s) - medicine , lipomatosis , magnetic resonance imaging , falx cerebri , calcification , atrophy , computed tomography , anatomy , pathology , radiology
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.