A Filipino male with encephalocraniocutaneous lipomatosis (Haberland's syndrome).

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.

• Authors: Iris Alessandra S Pardo, Marie Eleanore O Nicolas
• Journal: Journal of dermatological case reports (J Dermatol Case Rep) Vol. 7 Issue 2 Pg. 46-8 (Jun 30 2013) ISSN: 1898-7249 [Print] Poland
• PMID: 23858340 (Publication Type: Case Reports)