Porokeratotic eccrine ostial and dermal duct naevus with dermatomal trunk involvement: literature review and report on the efficacy of laser treatment

Abstract

Porokeratotic eccrine ostial and dermal duct naevus (PEODDN) is a rare, benign hamartomatous malformation involving the eccrine sweat duct. The existence of filiform keratinous plugs that represent cornoid lamellae overlying dilated infundibula of eccrine ducts is a distinctive feature and the presence of associated abnormal dermal ducts is frequent. We report a patient with PEODDN who exhibited lesions on the left side of her chest. Cases of PEODDN reported in the literature are reviewed. Our experience in treating this patient with ultrapulsed carbon dioxide laser is also presented.

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

“…It has been suggested that involvement of the epidermis with sparing of the adnexa may be used as a criterion in favor of a diagnosis of linear porokeratosis. 9,21 As several biopsies and multiple sections may be needed to differentiate linear porokeratosis and PAON, cases of PAON may have been missed because of an apparent lack of eccrine duct involvement. In addition, cornoid lamellae are well known to occur over adnexa in conventional porokeratosis.…”

“…The ultrapulsed carbon-dioxide laser has been used, but multiple treatments may be required 22 and the papules may recur. 21 Simple surgical excision should be reserved for cases with limited involvement, and the plaques can recur despite excision. 7 Topical corticosteroids, calcipotriol ointment, phototherapy, and cryotherapy have proven ineffective.…”

“…6 involving the proximal extremities, trunk, buttocks, neck, or a combination of these has been reported in 13 of 39 cases of PEODDN. 4,6,8,11,12,15,20,21,[23][24][25][26]29,31 Of the two cases of PEHFN, one had localized acral involvement 3 and the other had widespread bilateral involvement. 3,4 The clinical and histologic characteristics of our 5 cases of PAON and 13 previously reported widespread cases of PEODDN and PEHFN are summarized in Table I (available online at http://www.…”

“…10,11,17,[19][20][21][22]40 With time, the hyperkeratotic appearance of verrucous papules and plaques may diminish on the arms and legs, but persists on the palms and soles. 6,24 Similarities of our cases to previous reports include congenital onset, a distribution favoring the distal extremities and following Blaschko lines, and occasional progression in childhood to young adulthood, with persistence into adult life.…”

“…There have been two prior reports of PEHFN 3,4. Of the 39 total cases of PEODDN, 2 had onset of the disease at birth,1,[5][6][7][8][9]11,13,14,16,18,21,23,24,26, 29-31,33,34,36,39,41 6 had onset in the first few years of life…”