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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.
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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.

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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.

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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).

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