ABSTRACT
Porokeratotic adnexal ostial nevus may be misdiagnosed as wart based on clinical appearance and morphology. An accurate diagnosis through skin biopsy is crucial, as is the inclusion of rare disorders such as porokeratotic adnexal ostial nevus in the differential diagnosis.
ABSTRACT
El Nevo Poroqueratósico del Ostium y el Ducto Dérmico Ecrinos (NPODDE), es un raro hamartoma benigno de los conductos de las glándulas sudoríparas ecrinas, puede presentarse desde el nacimiento o también en edades posteriores. Su etiología plantea una alteración en la queratinización debido a una mutación somática en el gen GJB2 que codifica para una proteína de unión gap. Esta mutación también está relacionada con el síndrome KID por lo cual la asesoría genética es crucial en estos pacientes. Clínicamente puede presentarse como hoyuelos hiperqueratósicos en palmas y plantas que normalmente son asintomáticos. El diagnostico se confirma con la histopato-logía que muestra una laminilla cornoide sobre el conducto ecrino subyacente. La entidad es benigna y de difícil tratamiento siendo refractaria a varias modalidades terapéuticas. Se presenta un caso de un paciente adulto masculino con lesiones típicas en palmas y plantas, a quien con la biopsia de piel se le confirmó el diagnóstico de NPODDE. Dado la baja frecuencia de esta condición el objetivo de este artículo radica en actualizar los aspectos más relevantes de esta entidad.
Porokeratotic Eccrine Ostial and Dermal Duct Nevus (PEODDN) is a rare benign hamartoma of eccrine sweat gland ducts, it can present from birth or also at later ages. Its etiology implies an alteration in keratinization due to a somatic mutation in GJB2 gene, that codes for a gap junction protein. This mutation is also associated with KID syndrome so genetic counseling for parents is crucial. Clinically it can present as keratotic pits in palms and soles that are usually asymp-tomatic. The diagnosis is confirmed by histopathology that shows a cornoid lamellae on the underlying eccrine duct. The entity is benign and the treatment is difficult, being refractory to seve-ral therapeutic modalities. We present a case of a male adult patient with typical lesions on palms and soles, who was diagnosed with PEODDN by skin biopsy. Given the low frequency of this con-dition, the objective of this article is to update the most relevant aspects of this entity.
Subject(s)
Humans , Male , Adult , Skin Diseases/pathology , Porokeratosis/pathology , Nevus, Intradermal/pathology , Eccrine Glands/pathology , Skin Diseases/diagnosis , Porokeratosis/diagnosis , Nevus, Intradermal/diagnosis , HamartomaABSTRACT
Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare eccrine hamartoma, with treatment generally being unsatisfactory. The unique features of PEODDN presented include bilateral and facial lesions, and extensive body involvement. Management with CO2 laser was successful, and follow-up will be necessary to monitor for recurrent lesions.
