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 disorder of keratinization, involving acrosyringium. Most cases were congenital, and showed linear distribution of multiple hyperkeratotic comedo-like papules and plaques located on acral extremities. Adult-onset cases were extremely rare in the English literatures and there have not been any reported cases in the Korean literatures. Moreover, there has been no report describing the dermoscopic features of PEODDN so far. So, we here report a rare case of adult-onset PEODDN with its dermoscopic finding and review of the relevant literatures.
Subject(s)
Adult , Humans , Dermoscopy , Extremities , Keratins , NevusABSTRACT
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.
ABSTRACT
Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is an uncommon, benign dermatosis and is characterized by asymptomatic grouped keratotic papules and plaques with a linear pattern on the extremities. Various treatments, including topical steroids, topical calcipotriol, topical 5-fluorouracil, retinoid, cryotherapy, and carbon dioxide laser ablation have been tried for PEODDN, but the results were unsatisfactory. Recently, topical photodynamic therapy (PDT) has been shown to be effective for various cutaneous disorders. We report a case of PEODDN showing partial remission with PDT using topical 5-aminolevulanic acid in a 4-year-old girl.
Subject(s)
Calcitriol , Cryotherapy , Extremities , Fluorouracil , Lasers, Gas , Nevus , Photochemotherapy , Child, Preschool , Skin Diseases , Steroids , TriazenesABSTRACT
Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare, benign congenital hamartoma involving the eccrine sweat duct. Histologically, the distinctive feature of PEODDN is the existence of keratinous plugs that represent cornoid lamellae overlying dilated infundibula of eccrine ducts. Lesions are typically asymptomatic papules or plaques that are located on the distal extremities, including palms, fingers and soles. We report a case of a 7-year old girl with PEODDN who exhibited lesions on the left knee and the perianal region.
Subject(s)
Child , Female , Humans , Extremities , Fingers , Hamartoma , Knee , Nevus , SweatABSTRACT
Porokeratotic eccrine ostial and dermal duct nevus is considered to be a rare congenital harmatoma derived from eccrine duct. It is not associated with other congenital anomalies, though the cooccurence with linear psoriasis has been noted. We report the case of a 38-year-old woman with congenital porokeratotic eccrine ostial and dermal duct nevus associated with milia. She had asymptomatic multiple keratotic papules with keratotic plugs on the right foot.
Subject(s)
Adult , Female , Humans , Foot , Nevus , PsoriasisABSTRACT
Porokeratotic eccrine ostial and dermal duct nevus is considered to be a rare congenital hamartoma of eccrine duct origin. Histopathologic studies showed multiple cornoid lamella-like parakeratotic columns, which were associated with hyperplastic eccrine ostia and distal sweat ducts. I describe the case of a 20-year-old man with childhood-onset porokeratotic eccrine ostial and dermal duct nevus. He had a 10-year history of multiple keratotic papules and comedo-like pits on the thenar area and thumb of the right hand. But in my case, immunohistochemical studies for carcinoembryonic antigen showed no association with eccrine ostium and adjacent cornoid lamella-like structures.
Subject(s)
Humans , Young Adult , Carcinoembryonic Antigen , Hamartoma , Hand , Nevus , Sweat , ThumbABSTRACT
We report a case of porokeratotic eccrine ostial and dermal duct nevus in a 28-year old man. The skin lesions, present since birth, were multiple keratotic papules and punctate pits on the palms, soles and heels bilaterally. Histopathologically, the lesion was characterized by dilatation and hyperplasia of the eccrine sweat ducts with parakeratotic plugs related to sweat gland pores. This entity should be considered the differential diagnosis of other types of porokeratosis and comedo-like keratosis on palms and soles.
Subject(s)
Diagnosis, Differential , Dilatation , Heel , Hyperplasia , Keratosis , Nevus , Parturition , Porokeratosis , Skin , Sweat , Sweat GlandsABSTRACT
A 6-year-old boy with porokeratotic eccrine ostial and dermal duct nevus on the left sole is reported. The patient was born with keratotic plugs, each measuring 1-2 mm in diameter, on the left sole. Light-microscopic examination revealed epidermal invagination and cornoid lamella, which was found to be connected with hyperplastic intraepidermal and dermal eccrine duct. The present case can be histologically differentiated from nevus comedonicus and punctate porokeratotis.