Nevo poroqueratósico del ostium y el ducto dérmico ecrinos: reporte de caso / Porokeratotic eccrine ostial and dermal duct nevus: case report

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.

Case for diagnosis. Ulcerated intradermal nevus simulating Sister Mary Joseph's nodule

Abstract: We report a case of a 76-year-old patient with a history of recent weight loss and ulcerated umbilical nodular lesion. Initially, we considered the diagnostic hypothesis of Sister Mary Joseph's nodule. However, histopathological evaluation revealed that it was an ulcerated intradermal nevus. We perform a brief review of umbilical nodules.

Pruritic porokeratotic peno-scrotal plaques: Porokeratosis or porokeratotic epidermal reaction pattern? A report of 10 cases.

Background: Porokeratosis restricted to the genital region is rare with few cases described in the literature. Cases of porokeratosis restricted to the genital region are similar to plaque type of porokeratosis of Mibelli seen elsewhere on the body. We encountered 10 young males with pruritic plaques restricted to the peno-scrotal region, which clinically were not diagnosed as porokeratosis, but on biopsy revealed multiple cornoid lamellae, some of which were seen to arise from eccrine and follicular structures. Aims: The aim of this study is to study lesions restricted to the peno-scrotal region in males, which on biopsy showed cornoid lamellae suggestive of porokeratosis. Methods: Retrospective analysis of available data of patients who were rendered a histological diagnosis of genital porokeratosis. The database consisted of biopsies received in private consultation by the first author in the period January 2000 to March 2013. Results: Ten young men, 8 in their third decade, presented with pruritic plaques restricted to the peno-scrotal region of variable duration. The lesions were well-demarcated on the penis, but ill-defined with a rough granular surface on the scrotum. None of patients were diagnosed clinically as porokeratosis. The lesions were poorly responsive to topical steroid/antifungal treatment, but two patients showed partial improvement with oral isotretinoin. Biopsy in nine patients revealed multiple cornoid lamellae involving epidermis (6) and adnexal structures (3). One patient had a single cornoid lamella. Conclusion: The clinical and histological presentation of these patients is different from typical genital porokeratosis described in the literature and we postulate that these patients have an unusual porokeratotic reaction pattern of the epidermis with multiple cornoid lamellae.

Signo del tambaleo: una nueva clave en el diagnóstico dermatoscópico / Wobble sign syndrome: a new clue in dermatoscopic diagnosis

Los criterios dermatoscópicos clásicos, usados en los distintos métodos, son estáticos y evaluados por su presencia o ausencia. El "signo del tambaleo", a diferencia de los anteriores, es un criterio dermatoscópico dinámico y la forma de objetivizarlo también. Si al examinar una lesión pigmentada apoyamos el dermatoscopio sobre la misma y lo movemos horizontalmente, paralelo a la superficie cutánea, estamos agregando al exámen clásico un enfoque dinámico. En aquellos casos que exista un componente papular, la parte superior de la lesión pigmentada se pegará al dermatoscopio y seguirá su movimiento hacia uno u otro lado y la parte inferior no. El signo del tambaleo es un nuevo criterio dinámico para mejorar el diagnóstico clínico de las lesiones pigmentadas

Pigmented lesion of the oral cavity with eight years follow-up

A 27 year old white female with a pigmented lesion on the anterior portion of the mandibular gingival is described. A prophylactic excisional biopsy was performed in conjunction with a gingival graft. A gingival graft was performed to accelerate the healing process and reduce post operative discomfort. An intradermal nevus was diagnosed. The number of documented oral nevi in the literature now exceeds 200 and approximately half of them are histologically of the intradermal type. If early detection of thin oral melanomas is to be achieved, all pigmented oral cavity lesions should be viewed with suspicion.

Síndrome de Buschke-Ollendorf / Buschke-Ollendorf syndrome

Se presentan dos casos de síndrome de Buschke-Ollendorf (SBO) en una familia; la madre con una forma completa del síndrome y el hijo con una forma incompleta, sin osteopoiquilosis. Se realiza la descripción clínica de la entidad, de su histopatología y de los diagnósticos diferenciales. Motiva esta presentación el ser un síndrome poco frecuente