Porokeratotic eccrine ostial and dermal duct nevus

Abstract

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare variant of porokeratosis with characteristic histological feature of cornoid lamella involving the acrosyringium. We report a classic case of a 20-year-old male, who clinically presented to us with keratotic papules and plaque with pits, few having comedo like plugs, on right palm and sole since 1 year of age. A punch biopsy from palm was diagnostic as well as confirmatory showing cornoid lamella involving an eccrine duct which is the characteristic histopathological feature of PEODDN.

Hyperkeratotic papules following Blaschko lines in a 4‐year‐old boy

“…The involvement of Blaschko's lines implies mosaicism may play a role, and at least in some cases, PEODDN has been demonstrated to be a mosaic manifestation of the keratitis‐ichthyosis‐deafness (KID) syndrome, being caused by a somatic mutation in the gene GJB2 . Other medical conditions have been associated with PEODDN, including hyperthyroidism, sensory polyneuropathy, deafness, developmental delay, and squamous cell carcinoma …”

“…They are asymptomatic or mildly pruritic, and are predominately located on the palms and soles . The differential diagnosis of PEODDN includes porokeratosis plantaris, inflammatory linear verrucous epidermal nevus, nevus comedonicus, linear epidermal nevus, linear psoriasis, linear porokeratosis, lichen planus linearis, linear Darier's disease, and forms of ichthyosis with a Blaschkoid distribution, such as Conradi syndrome and CHILD syndrome . Histopathology is diagnostic, showing cornoid lamellae with the involvement of acrosyringia, usually associated with the dilation of eccrine duct .…”

“…3 Other medical conditions have been associated with PEODDN, including hyperthyroidism, sensory polyneuropathy, deafness, developmental delay, and squamous cell carcinoma. 4 The characteristic lesions of PEODDN are punctate pits or pitted papules in a linear arrangement. They are asymptomatic or mildly pruritic, and are predominately located on the palms and soles.…”

Porokeratotic eccrine ostial and dermal duct nevus

“…[ 2 ] Several case reports have described PEODDN associated with conditions such as hyperthyroidism and sensory polyneuropathy, breast hypoplasia, Bowen disease, deafness and development delay, seizure disorder, hemiparesis, scoliosis, alopecia, onychodysplasia, and squamous cell carcinoma. [ 9 ]…”

Unilateral punctate porokeratosis - Case report

“…It is also worth keeping in mind porokeratotic eccrine ostial and dermal duct nevus (PEODDN), a rare congenital hamartoma of eccrine origin, which may be clinically indistinguishable from PP, although on histological examination it shows multiple cornoid lamella-like parakeratotic columns, associated exclusively with the eccrine duct and ostia. 8 PEODDN is considered by some as a rare variant of porokeratosis. …”