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© 2013 The Authors. doi: 10.2340/00015555-1515 Journal Compilation © 2013 Acta Dermato-Venereologica. ISSN 0001-5555 Porokeratotic eccrine ostial and dermal duct naevus (PEODDN) (porokeratotic eccrine naevus) is an uncommon naevus characterized by a hyperkeratotic streak or hyperkeratotic streaks following Blaschko’s lines (1–3). Histopathological features include cornoid lamellae protruding from dilated eccrine ducts. PEODDN is a mosaic form of keratitis-ichthyosis-deafness (KID) syndrome caused by a mutation in GJB2 encoding a gap junction protein connexin-26 (Cx26) (3). Connexin proteins are components of gap junctions of aqueous pores, which allow intercellular exchanges of ions and small molecules (4). Gap junctions mediate intercellular communications in various physiological processes, including cell growth and differentiation. Connexin proteins assemble into hemichannels (connexons) and intercellular channels (gap junctions) (5). The mutation in GJB2 causes various disorders, including KID syndrome, Vohwinkel syndrome (VS), BartPumphrey syndrome (BPS), and non-epidermolytic palmoplantar keratoderma (NEPPK) with deafness. KID syndrome is associated with the aberrant function of gap junction (5). VS, BPS and NEPPK with deafness are related to the decreased function (5). Aberrant expression of K10, K14 and loricrin were illustrated in 2 cases of PEODDN (3), although proper expression of keratins and filaggrin were shown in cases of NEPPK with deafness (6). The aberrant function of gap junction would induce abnormal expression of keratins and keratinization-associated proteins. The theory prompted us to investigate keratins and filaggrin expression in our case of bilateral systematized PEODDN.

Porokeratotic Eccrine Ostial and Dermal Duct Naevus and Aberrantly Regulated Keratinization