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Research Article | Volume 7 Issue 2 (None, 2013) | Pages 46 - 48
A Filipino male with encephalocraniocutaneous lipomatosis (Haberland’s syndrome)
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Under a Creative Commons license
Open Access
PMID : PMC3710677
Received
Feb. 27, 2012
Published
June 30, 2013
Abstract

Background:Encephalocraniocutaneous lipomatosis (ECCL), also known as Haberland’s Syndrome, is a sporadically occurring neurocutaneous syndrome with no gender or race predilection. ECCL patients present with a broad spectrum of clinical manifestations, often in a unilateral distribution. The hallmark of ECCL is the nevus psiloliparus, a soft, bulging, lipomatous scalp lesion, with associated alopecia.Main observations: We describe a case of a 2-month-old Filipino male with a soft, ill-defined mass with associated alopecia on the fronto-parietal scalp. Biopsy revealed findings consistent with a nevus psiloliparus. The patient also presented with a lipomatous nodule on the right temple, as well as choristomas and a coloboma on the right eye. He had no history of seizures and development was at par with age.Conclusion:Recognition of ECCL is important in order to work-up the patient for concomitant problems, such as central nervous system and cardiac anomalies, and employ a multidisciplinary approach in the management of these patients.

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