Review ArticleFamilial Cases of PENS: A Review of the LiteratureSantos Silva, Leandro F. MD; Miroux-Catarino, Alexandre MD; Pimentel, Bernardo V. MD; Amaro, Cristina S. MD; Viana, Isabel F. MDAuthor Information Department of Dermatology, Hospital de Egas Moniz, Lisbon, Portugal. Correspondence: Leandro F. Santos Silva, Hospital de Egas Moniz, Rua da Junqueira 126, 1349-019 Lisboa, Portugal (e-mail: email@example.com). The authors declare no conflicts of interest. The American Journal of Dermatopathology: August 2020 - Volume 42 - Issue 8 - p 593-596 doi: 10.1097/DAD.0000000000001625 Buy Metrics Abstract Papular epidermal nevus with “skyline” basal cell layer (PENS) is a keratinocytic nevus that can occur sporadically or has a familial transmission. There are 5 families reported with PENS, in which there are 2 family members affected with each case. We present the sixth familial case, with the peculiarity of being the first time in which there are 3 family members with PENS, while reviewing the other cases described until now. In addition, we present a new histopathological finding, an inflammatory lichenoid infiltrate on the upper dermis in PENS lesions. This finding could be the result of trauma to the biopsied lesion, or it may represent a new inflammatory histological variant. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.