ABSTRACT
Poroceratose de Mibelli é uma genodermatose disceratósicade forma crônica, progressiva e rara, comrisco de evolução para malignidade. É de transmissãoautossômica dominante e a patogenia é ainda desconhecida.O caso clínico mostra um paciente com 78anos de idade, com 75 anos de evolução de Poroceratosede Mibelli com lesões gigantes, exuberantes ehistória médica familiar da genodermatose. As lesõesda poroceratose de Mibelli são assintomáticas e maiscomumente encontradas nas extremidades, preferencialmentedorso das mãos e pés. Pode apresentar progressãoe regressão espontânea e ocasionar cicatrizeslevemente atróficas. O diagnóstico é clínico-histológico,apresentando a lamela coróide no histológico. A policeratosede Mibelli deve ser tratada para evitar a transformaçãoem células escamosas, doença de Bowen oucarcinoma basocelular.
Porokeratosis of Mibelli is a chronic, progressiveand rare genodermatosis disceratosica, that has therisk to become a malignant tumor. Its transmissionis mainly autosomal and the pathogenesis is stillunknown. The clinic case shows a patient with 78years, 75 years of those with evolving Porokeratosisof Mibelli, huge injuries, and familiar historic ofgenodermatosis. The injuries from the Porokeratosisof Mibelli are asymptomatic and generally found inthe ends of the body, preferably in the back of thehand or feet. The injuries can increase or diminishspontaneously and lead to scars a little atrophic.The diagnosis is clinical-histological, and it is representedby a choroid lamella in the histological. ThePorokeratosis of Mibelli may be treated to avoid thetransformation into squamous cells, Bowen diseaseor basal cell carcinoma.
ABSTRACT
Porokeratosis of Mibelli, a chronic heritable disorder of the skin, presents with characteristic histopathological features including cornoid lamella as a result of faulty keratinization. Amyloid deposits are characterized by the accumulation of ultrastructually fibrillar material and have been observed in inflammatory and tumoral skin diseases. Some cases of amyloid deposits in the upper dermis in various subtypes of porokeratosis have been reported, but no porokeratosis of Mibelli reports are available in Korea. We report a case of a 26-year-old woman with three verrucous hyperkeratotic plaque lesions on her right popliteal fossa with histological feature of hyperkeratosis, cornoid lamella, and dermal amyloid deposits.
Subject(s)
Adult , Female , Humans , Amyloid , Dermis , Keratins , Korea , Plaque, Amyloid , Porokeratosis , Skin , Skin DiseasesABSTRACT
Porokeratosis is a disorder of keratinization showing a well-defined lesion with a hyperkeratotic ridge on the border that contains the coronoid lamella. We report familial (autosomal dominant with reduced penetrance) disseminated plaque type (Mibelli's type) porokeratosis in a father and son. In the father, there were multiple horns and a large squamous cell carcinoma in a large lesion over the perianal region that reached up to the squamo-columnar junction of the anal mucosa and even invaded the anal sphincteric muscles. Disseminated lesions of the Mibelli's type, development of horns, and malignancy in this unusual location have not been previously reported.
ABSTRACT
Porokeratosis is a disorder of epidermal keratinization that is histologically characterized by the presence of cornoid lamella. Many treatment modalities have been used, such as, surgical excision, cryosurgery, CO2 laser, oral etretinate, topical 5-fluorouracil and keratolytics. However, universal treatment guidelines have never been established. We tried 5% imiquimod cream on three patients who have porokeratosis of Mibelli which are intractable to other previous treatment modalities. Two of them showed a marked and moderate improvement, respectively. But the third patient exhibited only slight response. Herein we report our treatment experience and review the related literature.
Subject(s)
Humans , Acitretin , Cryosurgery , Etretinate , Fluorouracil , Lasers, Gas , PorokeratosisABSTRACT
We report a case of a 44 year-old man with porokeratosis of Mibelli, who presented with a 10 year history of a 1 cm diameter, well-circumscribed, erythematous, scaly patch with an elevated border on his glans penis. Histologic examination revealed a typical cornoid lamella and absence of an underlying granular layer. The localized type of porokeratosis of Mibelli is most commonly found on the limbs: however, the face, lips, mucosal surfaces, or genital area may instead be involved. We report a case of porokeratosis of Mibelli confined to the glans penis, a very rare site.
Subject(s)
Adult , Humans , Male , Extremities , Lip , Penis , PorokeratosisABSTRACT
There have been several reports of more than one type of porokeratosis occurring in the same family or the same individual. We hope to support the view of different phenotypic expressions of a common genetic aberration by describing an additional case of porokeratosis of Mibelli on the perianal area and DSAP on the face, forearms occurring in a 45-year-old man.
Subject(s)
Humans , Middle Aged , Actins , Forearm , Hope , PorokeratosisABSTRACT
We report a case of squamous cell carcinoma arising in porokeratosis of Mibelli in a 32-year-old male who presented with a large, slowly extending, erythematous patch with central ulceration on the left lateral side of the neck. Histopathologic examination of the periphery of the patch and central tumor showed porokeratosis and squamous cell carcinoma, respectively.