Article |

Picture of the Month

Pierre Bitoun, MD; Stephen Gellis, MD; Murray Feingold, MD
Am J Dis Child. 1982;136(12):1085-1086. doi:10.1001/archpedi.1982.03970480051011.
Text Size: A A A
Published online

Denouement and Discussion 

Encephalocraniocutaneous Lipomatosis 

Manifestations  Encephalocraniocutaneous lipomatosis (ECCL) is a congenital neurocutaneous syndrome that consists of skin lesions, ophthalmologic malformations, and cerebral abnormalities. The skin lesions appear as extensive (4 to 8 cm), raised, hairless, soft tumors, which are usually unilateral and involve the frontoparietal area of the scalp, with smaller lesions (3 to 5 mm) located around the eyes and cheek. Histologically, they are hamartomas of fat and connective tissue. Fleshy tumors (choristomas) are present on the sclerae. The CNS abnormalities consist of ipsilateral cerebral atrophy, dilated ventricles, porencephaly, varying degrees of mental retardation, and seizures that usually begin in infancy. Clinically, this syndrome can be confused with the linear nevus sebaceous syndrome, but the histologic findings of ECCL are different.

Genetics  The mode of inheritance is not known.

Treatment  Ophthalmologic, dermatologic, and neurologic examinations, including computed tomographic scans, should be performed. Neurosurgery may be indicated because of


Sign In to Access Full Content

Don't have Access?

Register and get free email Table of Contents alerts, saved searches, PowerPoint downloads, CME quizzes, and more

Subscribe for full-text access to content from 1998 forward and a host of useful features

Activate your current subscription (AMA members and current subscribers)

Purchase Online Access to this article for 24 hours

First Page Preview

View Large
First page PDF preview





Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).
Submit a Comment


Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

Related Content

Customize your page view by dragging & repositioning the boxes below.