Porokeratotic Eccrine Ostial and Dermal Duct Nevus of Late Onset: More Frequent than Previously Suggested?

Abstract

We describe the case of a 10-year-old boy with a porokeratotic eccrine ostial and dermal duct nevus (PEODDN) of late onset. The patient had an 8-year history of multiple keratotic papules on the dorsal surface, and multiple yellowish pitting lesions on the plantar surface of the right foot. Light-microscopic studies of both lesions showed multiple comoid lamella-like parakera-totic columns, which exclusively arose over eccrine sweat ducts in which the acrosyringium was dilated. Although PEODDN is considered to be a congenital hamartoma of eccrine origin, a review of the literature showed us that the frequency of a late-onset variant may be as high as 26%.

Unilateral linear punctate palmoplantar keratoderma

“…Our patient is also presented with an asymptomatic, tiny, hyperkeratotic punctate papules on the palmoplantar surface with unilateral left sole and left palm involvement without any associated diseases and we had made the differential diagnosis of linear PPPK, [1,2] linear punctate porokeratosis, [9] porokeratotic eccrine ostial and dermal duct naevus (PEODDN), [10,11] arsenical keratosis, [12] striate PPK, [13,14] Naegeli-Franceschetti-Jadassohn syndrome, [15] and punctate accentuated keratosis. [16] Above differential had been ruled out by their classical clinical presentation and histopathological features [ Tables 1 and 2].…”

“…[16] Above differential had been ruled out by their classical clinical presentation and histopathological features [ Tables 1 and 2]. [1,2,[9][10][11][12][13][14][15][16] Treatment of keratodermas is generally symptomatic like saltwater soaks, paring, topical keratolytics to specific therapies such as systemic retinoids, psoralens and ultraviolet A (PUVA), and re-PUVA. [2,8] When specific therapy does not works, then patient can be considered for reconstructive surgery with total excision of the hyperkeratotic skin followed by grafting.…”

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

“…Thirty two cases of PEODDN have been reported in the English-language literature to date, 1,2,5-32 with 9 additional reports in the French, [33][34][35][36][37] Spanish, 38,39 German, 40 (early onset), 2,19,20,25,35,40 and 9 in the teenaged years and adulthood (late onset). 10,12,15,17,22,27,28,32,35,37,38 Lesions typically present as asymptomatic keratotic to verrucous papules or plaques and multiple punctate pits filled with a comedo-like keratin plug.…”

“…Most patients have relatively localized involvement of an acral site, with 24 of 39 reported cases involving the palms/hands, soles/feet, or both. 1,2,5,7,9,10,13,14,16,18,19,22,27,28,30,[32][33][34][35][36][37][38][39][40][41] The vast majority of PEODDN case reports (32 of 39) describe unilateral involvement, with 7 having bilateral distribution. 6 involving the proximal extremities, trunk, buttocks, neck, or a combination of these has been reported in 13 of 39 cases of PEODDN.…”

“…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.…”