Porokeratotic eccrine ostial and dermal duct nevus

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Porokeratotic eccrine ostial and dermal duct nevus
Other namesPEODDN
Specialty Dermatology

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]

Contents

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]

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. [9]

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. [6]

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. [10] Topical steroids, retinoids, phototherapy, electrocautery, keratolytics, and cryotherapy are examples of modalities that have not demonstrated any encouraging outcomes. [2] [11]

See also

Related Research Articles

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Eccrine angiomatous hamartoma (EAH), first described by Lotzbeck in 1859, is a rare benign vascular hamartoma characterized histologically by a proliferation of eccrine and vascular components. EAH exists on a spectrum of cutaneous tumors that include eccrine nevus, mucinous eccrine nevus and EAH. Each diagnostic subtype is characterized by an increase in the number as well as size of mature eccrine glands or ducts, with EAH being distinguished by the added vascular component.

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<span class="mw-page-title-main">Nevus lipomatosus superficialis</span> Medical condition

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<span class="mw-page-title-main">Angioma serpiginosum</span> Medical condition

Angioma serpiginosum is characterized by minute, copper-colored to bright red angiomatous puncta that have a tendency to become papular.

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Traumatic anserine folliculosis is a curious gooseflesh-like follicular hyperkeratosis that may result from persistent pressure and lateral friction of one skin surface against another. Traumatic anserine folliculosis is caused by trauma. Topical keratolytics are the treatment of choice.

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<span class="mw-page-title-main">Trigeminal trophic syndrome</span> Medical condition

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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. 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. 1 2 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. 1 2 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.

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