February 1, 2008

Encephalocraniocutaneous Lipomatosis With Didymosis Aplasticopsilolipara

Author Affiliations

Copyright 2008 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2008

Arch Dermatol. 2008;144(2):266-268. doi:10.1001/archdermatol.2007.56

An 11-day-old boy born to nonconsanguineous Vietnamese parents presented to our pediatric dermatology outpatient department with a congenital linear scalp and face lesion. This soft, skin-colored plaque following the lines of Blaschko was devoid of terminal hair growth and extended from the left upper eyelid up to the ipsilateral vertex. At the vertex were 3 round, elevated, bald, bullous patches and 2 round scars and hair collars (Figure 1). On the upper eyelid, 2 soft, skin-colored papules were seen. Based on the clinical evaluation, encephalocraniocutaneous lipomatosis (ECCL) was suspected.

First Page Preview View Large
First page PDF preview
First page PDF preview