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Encephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report
Ashish Jagati1, Bela J Shah1, Rima Joshi1
1Department of Dermatology, B.J. Medical College, Civil Hospital, Ahmedabad, Gujarat, India.
Abstract:
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.

