Porokeratotic Eccrine and Hair Follicle Nevus: A Report of Two Cases and Review of the Literature

    January 2017 in “ Anais brasileiros de dermatologia/Anais Brasileiros de Dermatologia ”
    A.D. Agulló-Pérez, Miguel Ángel Resano-Abarzuza, Alicia Córdoba-Iturriagagoitia, J.I. Yanguas-Bayona
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    TLDR Porokeratotic eccrine and hair follicle nevus is a very rare skin disorder possibly caused by a GJB2 gene mutation.
    Porokeratotic eccrine and hair follicle nevus is an extremely rare disorder of keratinization involving eccrine glands and hair follicles, with only 9 cases previously reported. In 2009, the term porokeratotic anexial ostial nevus was introduced to include this condition and a related, more common disorder without follicular involvement, known as porokeratotic eccrine ostial and dermal duct nevus. Recent research suggested that both conditions might be caused by a mutation in the GJB2 gene, which is also linked to KID syndrome. This report presented 2 new cases and reviewed the existing literature in Spanish and English.
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