ABSTRACT
Las poroqueratosis son un grupo heterogéneo de trastornos de la queratinización epidérmica, de presentación infrecuente. Se caracterizan clínicamente por pápulas hiperqueratósicas que confluyen y forman placas anulares con un centro atrófico y bordes sobreelevados. Hay seis variantes clínicas. El examen histopatológico evidencia la característica laminilla cornoide. Se presenta el caso de una niña de 2 años con diagnóstico de poroqueratosis de Mibelli y respuesta parcial al tratamiento tópico con tretinoína al 0,025%, que actualmente continúa en seguimiento clínico (AU)
Porokeratosis is a heterogeneous group of disorders in epidermal keratinization. It is an infrequent entity characterized clinically by hyperkeratotic papules that converge forming annular plaques with an atrophic center and raised borders. There are six clinical variants. The histopathological examination evidences the typical cornoid lamella. We present a 2-year-old girl diagnosed with porokeratosis of Mibelli and partial response to topical treatment of tretinoin 0,025%, still under clinical control (AU)
Subject(s)
Humans , Female , Child, Preschool , Porokeratosis/diagnosis , Tretinoin/therapeutic use , Porokeratosis/pathology , Porokeratosis/drug therapy , Keratolytic Agents/therapeutic useABSTRACT
RESUMEN Se realizó la presentación de dos pacientes con poroqueratosis de Mibelli por ser una dermatosis poco frecuente, por lo que debe ser de interés para los profesionales de diferentes disciplinas, bien por relacionarse con el cáncer de piel o con disímiles enfermedades. Los pacientes estudiados fueron: paciente femenino de 62 años de edad, piel blanca y ocupación ama de casa. Fue remitida por presentar piel con fototipo cutáneo II, una lesión en placa de color amarillento, bordes elevados, hiperqueratósicos, centro atrófico de 1 cm de diámetro; localizada en cara ántero-externa de pierna derecha. El otro paciente, de 59 años, sexo masculino, piel blanca, y ocupación pescador. Solicitó los servicios por presentar piel fototipo cutáneo II lesión en placa de color amarillento, bordes elevados, hiperqueratósicos, centro atrófico de 2cm de diámetro, localizada en cara póstero-externa de antebrazo derecho. Se les realizó biopsia de ambas lesiones. El diagnóstico fue la poroqueratosis de Mibelli para la prevención del cáncer de piel. Ambos pacientes presentaron en común fototipo cutáneo II y otras manifestaciones cutáneas inducidas por la exposición solar. La terapéutica utilizada fue la extirpación quirúrgica, que resultó también el proceder diagnóstico Se orientaron medidas para la prevención del cáncer de piel con bloqueadores solares, y protegerse de los rayos solares con ropa adecuaday otros accesorios (AU).
ABSTRACT Two patients with Mibelli´s porokeratosis were presented because it is a few frequent dermatosis that might be of interest for professionals of different disciplines since it is related to skin cancer and to several other diseases. The first studied patients was 62 years old, female, white, and a housewife, remitted due to presenting skin with cutaneous phototype II, a yellowish lesion in plaque, risen, hyperkeratoid borders and atrophic center of 1 cm diameter, located in the anterior-external face of the right leg. The other patient was 59 years old, male, white, and a fisherman. He assisted the service presenting skin with cutaneous phototype II, a yellowish lesion in plaque, risen, hyperkeratoid borders and atrophic center of 2 cm diameter, located in the posterior-external face of the right forearm. A biopsy of both lesions was carried out. The diagnosis was Mibelli´s porokeratosis. Both patients presented a common cutaneous phototype II and skin manifestations induced by the sun exposition. The used therapeutic was the surgical removal, that also was the diagnosis procedure. Preventing skin cancer with sun blockers was oriented and also wearing adequate clothes and other accessories (AU).
Subject(s)
Humans , Male , Middle Aged , Skin Diseases , Skin Neoplasms/prevention & control , Porokeratosis/diagnosis , Porokeratosis/therapy , Photochemotherapy , Biopsy/methods , Carcinoma, Squamous Cell , Health Education , Patient Education as Topic , Risk Factors , Protective Factors , Health PromotionABSTRACT
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
Los angioqueratomas son lesiones vasculares relativamente infrecuentes que consisten en pápulas hiperqueratósicas rojo-violáceas. Éstas pueden ser únicas o múltiples, estar localizadas en un solo segmento corporal o ser generalizadas y estar o no asociadas a otras enfermedades subyacentes. El presente trabajo abordará en profundidad los angioqueratomas localizados y se complementará con una segunda parte en la que se tratarán los angioqueratomas generalizados.
Angiokeratomas are a relatively non-frequent group of vascular lesions that consist on hyperkeratotic red-violaceous papules. Lesions can be solitary or multiple, localized or generalized and may be associated or not with a systemic disease. The present work is a throughout review on localized angiokeratomas and will be complemented with a second part in which generalized angiokeratomas will be discussed.
Subject(s)
Humans , Angiokeratoma , Fox-Fordyce Disease , Porokeratosis , Hyperkeratosis, Epidermolytic , Skin Neoplasms , Tongue 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 porokeratosis of Mibelli coexistence with linear porokeratosis in a 28-year old male. He had multiple discrete reddish to brownish annular plaques with peripheral keratotic ridges on the left side of the neck and axilla, and such lesions extended in a linear fashion on the left side of the upper arm and posterior aspect of the lower extremity. The cutaneous lesions started on the left neck and axilla about 6 years prior to consultation. Two years after that, new lesions developed on the left side of the upper arm and lower extremity in a linear fashion. There was no family history of similar skin disorders. A histological examination showed cornoid lamellae in the horny layer. Hypogranulosis, dyskeratotic cells and perivascular mononuclear in-filtrations were also seen beneath the cornoid lamella.
Subject(s)
Humans , Male , Arm , Axilla , Lower Extremity , Neck , Porokeratosis , SkinABSTRACT
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.