Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address Please contact the publisher to request reinstatement.
We're unable to sign you in at this time. Please try again in a few minutes.
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Observation |

Acantholytic Dyskeratotic Epidermal Nevus

Olushola Akinshemoyin Vaughn, BA1; Molly A. Hinshaw, MD2,3; Joyce M. Teng, MD, PhD4
[+] Author Affiliations
1University of Wisconsin School of Medicine and Public Health, Madison
2Department of Dermatology, University of Wisconsin, Madison
3Dermatopathologist, Dermpath Diagnostics, Brookfield, Wisconsin
4Department of Dermatology, Lucile Packard Children’s Hospital at the Stanford University School of Medicine, Palo Alto, California
JAMA Dermatol. 2015;151(11):1259-1260. doi:10.1001/jamadermatol.2015.1663.
Text Size: A A A
Published online


This case report describes a possible solution to the challenge of clinically distinguishing acantholytic dyskeratotic epidermal nevus, often a congenital condition, from Darier disease, which is acquired.

Acantholytic dyskeratotic epidermal nevus (ADEN) is histologically similar to Darier disease (DD), and distinguishing the 2 entities may be challenging. We describe the case of an 18-month-old boy with congenital epidermal nevi, histological acantholytic dyskeratosis, and the presence of sarcoendoplasmic reticulum calcium transport ATPase 2 (SERCA2) protein by immunohistochemical (IHC) staining (Abcam PLC). We concluded that the best diagnosis was ADEN.

Figures in this Article

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

First Page Preview

View Large
First page PDF preview


Place holder to copy figure label and caption
Figure 1.
Child With Acantholytic Dyskeratotic Epidermal Nevus (ADEN)

Typical cutaneous findings in ADEN include verrucous papules and plaques, often following the lines of Blaschko. This patient had linear involvement of the left lower extremity.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.
Immunohistochemical Staining of Lesional Skin Using SERCA2

This photomicrograph demonstrates that lesional skin tested positive under sarcoendoplasmic reticulum calcium transport ATPase 2 (SERCA2) immunohistochemical stain (1:100 dilution; Abcam PLC), favoring a diagnosis of ADEN over Darier disease in our patient (original magnification ×400).

Graphic Jump Location




Also 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.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
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.


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

0 Citations

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

Related Content

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

Articles Related By Topic
Related Collections
PubMed Articles