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Karola Maria Stieler, MD; Susanne Astner, MD; Georg Bohner, MD; Natalie Garcia Bartels, MD; Hans Proquitté, MD; Wolfram Sterry, MD, PhD; Norbert Haas, MD, PhD; Ulrike Blume-Peytavi, MD, PhD

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Report of a Case

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.

Figure appears in full text version. Figure 1. Linear soft lesion extending from the vertex to the left eye and associated membranous skin defects (membranous aplasia cutis congenita) with hair collars form the didymosis aplasticopsilolipara. Note . . . [Full Text of this Article]

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