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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. It is probably transmitted by paradominant transmission.

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. 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. Plaques are frequently formed from the keratotic papules, and more advanced lesions typically disperse throughout Blaschko's lines. Although it is typically asymptomatic, moderate pruritus, hyperhidrosis, or anhidrosis may coexist with it.

Causes

It is unknown what causes PEODDN. The hypothesized pathomechanism of the entity appears to be genetic mosaicism and a possible eccrine or restricted epidermal aberration of keratinization. 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.

PEODDN has been linked in a number of case reports to disorders such sensory polyneuropathy and hyperthyroidism, breast hypoplasia, Bowen disease, alopecia, hemiparesis, scoliosis, deafness and development delay, seizure disorder, and squamous cell carcinoma.

Diagnosis

The foundation of diagnosis is histopathology; cornoid lamella with acrosyringia involved is pathognomonic for PEODDN. It is typically linked to eccrine duct dilatation. Differential diagnoses include inflammatory linear verrucous epidermal nevus, porokeratosis plantaris discreta, nevus comedonicus, linear psoriasis, linear epidermal nevus, spiny keratoderma, congenital unilateral punctate porokeratosis, linear porokeratosis, and porokeratosis of Mibelli.

Treatment

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. Topical steroids, retinoids, phototherapy, electrocautery, keratolytics, and cryotherapy are examples of modalities that have not demonstrated any encouraging outcomes.

See also

• Prominent inferior labial artery
• List of cutaneous conditions

References

1. Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
2. ^ Masferrer E, Vicente MA, Bassas-Vila J, Rovira C, González-Enseñat MA (Jul 2010). "Porokeratotic eccrine ostial and dermal duct naevus: report of 10 cases". J Eur Acad Dermatol Venereol. 24 (7): 847–51. doi:10.1111/j.1468-3083.2009.03498.x. PMID 19925595. S2CID 205589524.
3. ^ Bandyopadhyay, Debabrata; Saha, Abanti; Das, Dipti; Das, Anupam (2015). "Porokeratotic eccrine ostial and dermal duct nevus". Indian Dermatology Online Journal. 6 (2). Medknow: 117–119. doi:10.4103/2229-5178.153016. ISSN 2229-5178. PMC 4375756. PMID 25821735.
4. Cambiaghi, Stefano; Gianotti, Raffaele; Caputo, Ruggero (2007). "Widespread Porokeratotic Eccrine Ostial and Dermal Duct Nevus Along Blaschko Lines". Pediatric Dermatology. 24 (2). Wiley: 162–167. doi:10.1111/j.1525-1470.2007.00367.x. ISSN 0736-8046. PMID 17461816.
5. Zade, John; Jfri, Abdulhadi; Nabatian, Adam; Alajaji, Abdullah; Geller, Lauren; Khorasani, Hooman (2017). "Porokeratotic eccrine ostial and dermal duct nevus: a unique case treated with CO 2 laser". Clinical Case Reports. 5 (5): 675–678. doi:10.1002/ccr3.846. ISSN 2050-0904. PMC 5412763. PMID 28469874.
6. ^ Naraghi, Mona Masoumeh; Nikoo, Azita; Goodarzi, Azadeh (2013). "Porokeratotic Eccrine Ostial and Dermal Duct Nevus". Case Reports in Dermatological Medicine. 2013. Hindawi Limited: 1–3. doi:10.1155/2013/953840. ISSN 2090-6463. PMC 3834619. PMID 24307955.
7. 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. ISSN 1087-2108. PMID 21971275.
8. Easton, Jennifer A.; Donnelly, Steven; Kamps, Miriam A.F.; Steijlen, Peter M.; Martin, Patricia E.; Tadini, Gianluca; Janssens, René; Happle, Rudolf; van Geel, Michel; van Steensel, Maurice A.M. (2012). "Porokeratotic Eccrine Nevus May Be Caused by Somatic Connexin26 Mutations". Journal of Investigative Dermatology. 132 (9). Elsevier BV: 2184–2191. doi:10.1038/jid.2012.143. ISSN 0022-202X. PMC 3422696. PMID 22592158.
9. Pathak, Deeptara; Kubba, Raj; Kubba, Asha (2011). "Porokeratotic eccrine ostial and dermal duct nevus". Indian Journal of Dermatology, Venereology and Leprology. 77 (2). Medknow: 174–176. doi:10.4103/0378-6323.77457. ISSN 0378-6323. PMID 21393947.
10. Kumar, Piyush; Mondal, Avijit; Das, Anupam; Debbarman, Panchami; Mandal, RajeshKumar (2015). "Porokeratotic eccrine ostial and dermal duct nevus: A noteworthy presentation". Indian Dermatology Online Journal. 6 (2). Medknow: 130–131. doi:10.4103/2229-5178.153021. ISSN 2229-5178. PMC 4375761. PMID 25821740.
11. 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. ISSN 0190-9622. PMID 19664847.

Further reading

• Kumar, SSudheer; Kumar, KRam; Chenchaih, V; Usha, Grandhi; Ramana, PVenkata (2021). "Porokeratotic eccrine ostial and dermal duct nevus: A rare case report". Journal of Clinical and Scientific Research. 10 (4): 246. doi:10.4103/JCSR.JCSR_83_20. ISSN 2277-5706.
• 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. ISSN 0190-9622. PMID 10901725.

External links

• DermNet
• VisualDx

• Dermal and subcutaneous growths