Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a skin lesion that resembles a comedonal nevus, but it occurs on the palms and soles where pilosebaceous follicles are normally absent.[1] It is probably transmitted by paradominant transmission.[2]
Signs and symptoms
PEODDN is defined by the characteristic presentation of numerous punctate or keratotic papules on the extremities that develop during infancy or at birth.[3] While lesions are typically localized to the distal extremities, which are the most prevalent sites of occurrence, reports have also indicated broad involvement of the trunk, face, and proximal extremities.[4] Plaques are frequently formed from the keratotic papules, and more advanced lesions typically disperse throughout Blaschko's lines.[5] Although it is typically asymptomatic, moderate pruritus, hyperhidrosis, or anhidrosis may coexist with it.[6]
Causes
It is unknown what causes PEODDN.[3] The hypothesized pathomechanism of the entity appears to be genetic mosaicism and a possible eccrine or restricted epidermal aberration of keratinization.[7] Recent research suggests that PEODDN is a mosaic variant of keratitis ichthyosis deafness (KID) syndrome caused by a somatic mutation in GJB2, which encodes a gap junction protein called connexin-26.[8]
There are few choices for treatment. With time, some lesions may spontaneously flatten. Surgery may be a good option for small, isolated lesions. Laser therapy is a great technique because there is very little risk of pigmentary alterations and scarring, especially when using an ultra-pulse CO2 laser. Patients with PEODDN have demonstrated considerable cosmetic improvement with combined erbium/CO2 laser therapy.[10]Topical steroids, retinoids, phototherapy, electrocautery, keratolytics, and cryotherapy are examples of modalities that have not demonstrated any encouraging outcomes.[2][11]
^Wong, Jillian W.; Summers, Erika M.; Taylor, Mark B.; Harris, Ronald M. (2011-09-15). "Porokeratotic eccrine ostial and dermal duct nevus treated with a combination erbium/CO2 laser: a case and brief review". Dermatology Online Journal. 17 (9): 10. doi:10.5070/D36550J2TT. ISSN1087-2108. PMID21971275.
^Goddard, Deborah S.; Rogers, Maureen; Frieden, Ilona J.; Krol, Alfons L.; White, Clifton R.; Jayaraman, Anu G.; Robinson-Bostom, Leslie; Bruckner, Anna L.; Ruben, Beth S. (2009). "Widespread porokeratotic adnexal ostial nevus: Clinical features and proposal of a new name unifying porokeratotic eccrine ostial and dermal duct nevus and porokeratotic eccrine and hair follicle nevus". Journal of the American Academy of Dermatology. 61 (6). Elsevier BV: 1060.e1–1060.e14. doi:10.1016/j.jaad.2009.03.036. ISSN0190-9622. PMID19664847.
Sassmannshausen, Jeffrey; Bogomilsky, Jodie; Chaffins, Marsha (2000). "Porokeratotic eccrine ostial and dermal duct nevus: A case report and review of the literature". Journal of the American Academy of Dermatology. 43 (2): 364–367. doi:10.1067/mjd.2000.100536. ISSN0190-9622. PMID10901725.