Poroqueratosis de localización nasal. Reporte de un caso / Porokeratosis of nasal location. Case report

RESUMEN

RESUMEN La poroqueratosis es un trastorno de la queratinización, con herencia autosómica dominante en la mayoría de los casos, vinculada posiblemente a la presencia de un clon mutante de queratinocitos en expansión. Tiene diferentes formas clínicas de Mibelli, actínica superficial diseminada, lineal, puntiforme, palmoplantar y facial atípica. El diagnóstico se confirma con la histología, presentándose típicamente la laminilla cornoide, que no es patognomónica de esta afección. En el tratamiento de esta patología se utilizan desde agentes tópicos como emolientes, 5-fluorouracilo, calcipotriol, imiquimod y retinoides como terapia sistémica. Otras opciones terapéuticas descritas son extirpación quirúrgica, criocirugía y dermoabrasión cuando se trata de formas localizadas o únicas. Se decide reportar el caso de una paciente femenina con poroqueratosis de afección exclusiva del área nasal por su rara frecuencia y escasos reportes sobre esta forma de presentación en la actualidad.

ABSTRACT

ABSTRACT Porokeratosis is a disorder of keratinization, with AD inheritance in most cases; possibly linked to the presence of a mutant clone of expanding keratinocytes, in addition to the overexpression of the p53 gene that could be related to an oncogenic potential. This disorder has also been associated with factors such as UVR, immunosuppression cases such as HIV +, liver or kidney failure, transplantation of organs that would act as triggers of this disease. It differs from other pathologies due to the presence of a peripheral hyperkeratotic ring which is usually asymptomatic in half of the patients. Clinically it can occur in different ways porokeratosis de Mibelli, superficial actinic disseminated, linear, punctate, palmoplantar and atypical facial. Diagnosis is confirmed with histology, with the cornoid lamella typically present, which is not pathognomonic of this condition and consists of a parakeratotic keratinocyte column that extends from an invagination of the epidermis through the stratum corneum. The treatment to choose, will depend on the patient´s age; Size and location of the lesions, the aesthetic implications and the general condition of the affected person. They are used from topics such as emollients, 5-fluorouracil, calcipotriol, imiquimod. Retinoids are found as an option for systemic therapy. Another procedure considers surgical removal, cryosurgery and dermabrasion when dealing with localized or unique forms. We report a case of a 44-year-old female patient who presents with compatible dermatosis porokeratosis, affecting only the nasal area.