Poroqueratosis de Mibelli. Presentación de caso / Mibelli's porokeratosis. Presentation of case

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).

Disseminated superficial actinic porokeratosis: a case report

Disseminated superficial actinic porokeratosis [DSAP] is characterized by small, atrophic patches with distinctive keratin rims that occur on sun-exposed areas of the extremities, shoulders, and back. The diagnosis is based on the histopathologic finding of a cornoid lamella, absence of a granular layer, and often a thin epidermis. It is associated with exposure to ultraviolet radiation. We report a case of DSAP in our setting

Poroqueratosis genital: reporte de caso y revisi¢n de la literatura / Genital porokeratosis: case report and review of the literature

La poroqueratosis es un grupo de dermatosis raras que se producen debido a un trastorno del proceso de queratinizaci¢n. Hay diferentes formas cl¡nicas, pero caracter¡sticamente se presenta como p pulas delimitadas con centro ligeramente atr¢fico y borde elevado hiperquerat¢sico, pueden causar prurito. En la histopatolog¡a se puede observar las lamelas carnoides y la hipogranulosis como caracter¡sticas importantes. Se presenta el caso de un paciente var¢n de 32 a¤os de edad con lesiones en la regi¢n genital una localizaci¢n rara para esta enfermedad pero con cl¡nica e histopatolog¡a t¡picas, que fue tratado con crioterapia.

The porokeratosis is a rare dermatosis occurring due to a disorder of keratinization process. There are different clinical forms, but characteristically arises as papules delimited with center slightly atrophic and edge high hyperkeratotic, which can cause itching. The histopathology, you can see the lamellae carnoides and the hipogranulosis as important features. A case of a 32-year-old male patient is presented with lesions in the genital region, a rare location for this disease but with typical clinical and histopathology, which was treated with cryotherapy.

Case for diagnosis / Caso para diagnóstico

Porokeratosis is a primary keratinizing disorder of unknown etiology. This disorder is characterized by the presence of centrifugally enlarging hyperkeratotic plaques, associated with the histopathological hallmark of cornoid lamellae. Genital porokeratosis is extremely rare. No more than thirty cases have been reported in the literature, including only one case of linear porokeratosis confined to the genital area. This case report describes a patient with genital linear porokeratosis, who was successfully treated with cryotherapy. Over two years of follow-up, the lesion improved and there was no evidence of recurrence or signs of malignant transformation. Nevertheless, there is a need for long-term follow-up data on recurrence and malignant transformation.

A poroceratose é uma desordem primária de queratinização, de etiologia desconhecida. Apresenta-se com lesões eritemato-acastanhadas com crista periférica e centro normal ou atrófico, e está associada ao achado anatomopatológico de lamela cornoide. O envolvimento genital nessa doença é extremamente raro. Não mais que trinta casos têm sido descritos na literatura, sendo um desses casos de poroceratose linear localizado na área genital. Descrevemos um caso de poroceratose linear genital tratado com crioterapia. Durante dois anos de seguimento, houve melhora do aspecto das lesões, sem recorrência ou sinais de transformação maligna. Entretanto, um acompanhamento a longo prazo se faz necessário.

Poroqueratosis superficial diseminada de la cara / Disseminated superficial facial porokeratosis

La poroqueratosis exclusivamente facial es una presentación clínica inusual de la poroqueratosis superficial diseminada, con muy pocos casos reportados en la literatura. Se presenta el caso de una mujer de 29 años con 10 años de evolución de poroqueratosis superficial diseminada de la cara.

[Porokeratosis. A study about 6 cases]

Porokeratoses [PK] represent a less common group of dermatoses that are acquired or hereditary, of unknown pathogenesis, characterized b keratinization disorder. Different clinical forms have been identified and the most frequent are the Mibelli's porokeratosis [MP] and the Disseminated Superficial Actinic Prokeratosis [DSAP]. In this retrospective study, we analysed the observations of PK collected in the Dermatology Department of La Rabta Hospital over a 16-year period. Six cases of PK were collected: 4 females and 2 males, with no medical history with a mean-age of 42.7. Only a female patient had [PK] familial history. All our patients had typical clinical aspects with lesions in annular plaques, of atrophic center, surrounded by keratotic border. The histological aspect was consistent with PK, showing the typical cornoid slide. In our series, there are 3 cases of [MP] and 3 cases of [DSAP]. PK lesions usually appear during the childhood and the third and fourth decades for the DSAP. In MP, there is one or some large, unilateral, annular plaques. DSAP is characterized by numerous small annular lesions of the photo-exposed regions Their prognosis is on the whole, favourable but remains conditioned by malignant transformation. Treatment is difficult aiming at reducing that risk of degeneration

Poroqueratosis / Porokeratoses

Las poroqueratosis son enfermedades genéticamente determinadas, definidas como desórdenes específicos de la queratinización. Se han clasificado en cinco grandes variantes clínicas: la poroqueratosis actínica superficial diseminada, la poroqueratosis clásica de Mibelli, la poroqueratosis palmoplantar diseminada, la poroqueratosis lineal y la poroqueratosis punctata, caracterizadas histológicamente por la presencia de la lámina cornoide, una columna paraqueratósica densa, bien delimitada del resto de los corneocitos. Todas las formas de presentación tienen riesgo de transformación neoplásica. Los pacientes con poroqueratosis deben fotoprotegerse adecuadamente y evitar la exposición solar excesiva y deben tener un control dermatológico periódico para pesquisar precozmente lesiones neoplásicas.

Porokeratosis of Mibelli: an uncommon genodermatosis

Porokeratosis of Mibelli is an uncommon, inherited, autosomal dominant disorder characterized by disordered epidermal keratinization and by a predisposition to develop malignant transformation. A case of this rare disorder with classical presentation in a teen aged boy is reported here, which was managed with topical retinoid, superficial chemical peeling and sunscreen lotion

Poroqueratosis lineal / Porokeratosis lineal

Se presentan dos casos clínicos de poroqueratosis zoniforme de Mibelli, afectando dos miembros de una familia, madre e hijo, los cuales no respondieron a la terapeútica tópica con queratolíticos. Dada la infrecuencia de esta variedad clínica de porosqueratosis, se realiza una revisión y actualización sobre el tema

Poroceratose palmar e plantar disseminada com lesöoes de mucosa oral: relato de caso / Prokeratosis palmaris and plantaris disseminata with oral lesions: a case report

Os autrores apresentam caso de mulher com 56 anos portadora de poroceratose palmar e plantar disseminada, com lesöes da mucosa oral. Apesar de 46 anos de evoluçäo da doença e uma resposta negativa ao DNCB e testes intradérmicos para avaliaçäo da imunidade celular, näo apresenta cancerizaçäo de nenhuma lesäo cutâneo-mucosa. O uso de laser de CO resultou em regressäo das lesöoes irradiadas.