Poroqueratosis de Mibelli / Porokeratosis of Mibelli

Introducción: La Poroqueratosis de Mibelli es una entidad rara en nuestro país, incluida en el grupo de las genodermatosis, por ser un trastorno hereditario de la queratinización. Es de gran interés para varias ramas de la medicina: genética, dermatología, medicina interna entre otras. Caso clínico: Se presenta un caso en una familia portadora de esta enfermedad genética con carácter autosómico dominante, se identificaron 7 miembros afectados en 3 generaciones, sin complicaciones. El propósito fue confirmado a través del estudio histopatológico. Conclusiones: Aunque no es un problema relevante en el municipio y no tiene complicaciones que comprometan la vida de los pacientes, sí pueden verse afectados según la localización de las lesiones, por lo que es de gran importancia el asesoramiento genético a los miembros de la familia para evaluar el riesgo genético, para poder realizar la profilaxis y control de los miembros afectados y su descendencia(AU)

Introduction: Porokeratosis of Mibelli is a rare condition in our country. It is included in the group of genodermatoses, being as it is an inherited keratinization disorder. This skin condition if of great interest to several medical specialties, such as genetics, dermatology, internal medicine, and others. Clinical case: A case is presented of a family carrying this genetic disease of an autosomal dominant nature. Seven affected members were identified in three generations without any complications. The purpose was confirmed via histopathological examination. Conclusions: Although the study condition is not a relevant problem in the municipality and does not have complications threatening the lives of patients, these may be affected depending on the location of the lesions. Therefore, genetic counseling is of great importance, so that family members may evaluate the genetic risk, perform the corresponding prophylactic actions, and control affected members and their offspring(AU)

Dermatopathology of skin cancer in solid organ transplant recipients.

Skin cancers occur more frequently in solid organ transplant recipients relative to the general population. Transplant recipients are at particularly high risk of squamous cell carcinoma, with up to a 100-fold increase in the relative risk when compared to the nontransplanted population. This compares with a 10- to 16-fold increase in basal cell carcinoma for renal transplant recipients. An increased incidence of melanoma in transplant patients has also been reported. Other types of skin cancer associated with immunosuppression in transplant patients include Kaposi sarcoma, Merkel cell carcinoma, and posttransplant lymphoproliferative disorder. This review discusses the epidemiology and pertinent pathologic features of each of these tumors. A brief clinical management strategy is outlined. In addition, the contribution of viral induced carcinogenesis with respect to Kaposi sarcoma, Merkel cell carcinoma, and posttransplant lymphoproliferative disorder is discussed.

Multicentric squamous cell carcinoma over lesions of porokeratosis palmaris et plantaris disseminata and giant porokeratosis.

Porokeratosis is a specific disorder of keratinization that has five clinical types and shows a characteristic 'cornoid lamella' on histopathology. Malignant degeneration has been described in all forms of porokeratosis. To the best of our knowledge, this is the first Indian report of multicentric squamous cell carcinoma complicating porokeratosis.

Solar facial porokeratosis.

Porokeratosis is comprised of a heterogeneous group of disorders that are inherited in autosomally dominant fashion. It has a wide variety of manifestations. In the last ten years, we have treated cases of porokeratosis that don't fit into the well known categories of porokeratosis. For this reason, the present study was carried out. This study included 15 cases whose ages ranged from 19-37 years with a mean +/- SD of 26.93 +/- 4.52. There were 12 females and 3 males. The duration of the disease ranged from 1-10 years with a mean +/- SD of 4 +/- 2.85 years. Their lesions were located only on the face, mostly on the distal part of the nose and the surrounding perinasal area. The number of lesions on each patient ranged from 1-15, with a mean +/- SD of 4.73 +/- 4.35. The morphology of these lesions consisted of papules with a size range from 0.1-1 cm and a warty appearance. They were skin colored and surrounded by a keratotic rim in the form of a collar. The histopathology showed the picture of porokeratosis with cornoid lamellae and additional features characteristic of this disease. We concluded that facial solar porokeratosis is a variant of porokeratosis and is a disease of the nose of young females with a characteristic clinical and histopathological picture.

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.

Porokeratosis of Mibelli. Overview and review of the literature.

Porokeratosis of Mibelli is an uncommon dermatosis, which may be associated with immunosuppression and may undergo malignant transformation. Due to the wide range of clinical presentations, numerous classifications have evolved, resulting in some confusion. This article examines the classification and presentation of porokeratosis and, in particular, reviews the association with immunosuppression.

A report of 31 cases of porokeratosis at the National Skin Centre.

Porokeratosis is a well-recognised disorder of keratinization with distinctive clinical features and histological hallmark of cornoid lamella. There are at least 4 different clinical variants, with malignant transformation reported in almost all types of porokeratosis. This is a retrospective study on all cases of porokeratosis seen at the National Skin Centre, Singapore from 1990 to 1993. There was a total of 31 patients diagnosed to have porokeratosis during the study period. They can be classified into 4 main clinical variants: (1) disseminated superficial actinic porokeratosis (41.9%), (2) classical porokeratosis of Mibelli (35.5%), (3) porokeratosis palmaris, plantaris et disseminatum (9.7%), and (4) linear porokeratosis (12.9%). Our typical patient is in his/her early forties, who noticed asymptomatic porokeratotis lesion on sun-exposed skin. Various treatment modalities were used, with no one method being more superior to another. None of our patients had malignant transformation of pre-existing skin lesions during the short follow-up period from less than one year to three years. Patients should be advised to avoid excessive sunlight, to use sunscreen and go for periodic examination by a dermatologist with a view to close skin malignancy surveillance.