Porokeratotic Eccrine Duct and Hair Follicle Nevus (PEHFN) Associated with Keratitis-Ichthyosis-Deafness (KID) Syndrome
September 2010
in “
Pediatric dermatology
”
TLDR A 15-year-old with KID syndrome developed a rare skin condition called PEHFN.
The document reported a case of a 15-year-old woman with keratitis-ichthyosis-deafness (KID) syndrome who developed widespread porokeratotic eccrine and hair follicle nevus (PEHFN). PEHFN is a rare hamartomatous malformation characterized by cornoid lamellae overlying dilated eccrine ostia and involves hair follicle infundibula. This condition typically presents as multiple filiform keratotic spines in a linear arrangement, often on the distal extremities. The case highlighted the association between PEHFN and KID syndrome, suggesting a potential link between these rare conditions.
