Widespread Presentation And Spontaneous Regression Of Porokeratotic Eccrine Ostial And Dermal Duct Nevus

Abstract

CO2: carbon dioxide PEHFN: porokeratotic eccrine and hair follicle nevus PEODDN: Porokeratotic eccrine ostial and dermal duct nevus INTRODUCTION Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is an uncommon hamartomatous growth with disordered keratinization. The lesions typically appear on the limbs, often at birth or in early childhood, as linearly distributed papules and plaques. When involving the palms or soles, keratotic plugs are often seen. PEODDN is typically unilateral; however, bilateral involvement has been reported. Lesions often persist over time with rare reports of associated malignancies. Treatment is challenging and options include erbium/carbon dioxide (CO2) lasers, keratolytic agents, and surgical excision. Cases of PEODDN have been reported in association with deafness, polyneuropathy and hyperthyroidism, seizure disorder, and hemiparesis. Most patients are healthy, however, and these associations may be coincidental. Histologically, the lesions show epidermal hyperplasia with cornoid lamellae located over the eccrine ostia. PEODDN is believed to be caused by somatic mutation and mosaic expression of mutated GJB2 gene that encodes the gap junction protein connexin 26. The term porokeratotic adnexal ostial nevus has been suggested to describe both the PEODDN and the related lesion, porokeratotic eccrine and hair follicle nevus (PEHFN). We report 4 cases of PEODDN, 2 of which showed significant spontaneous regression.