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B B Wilson - One of the best experts on this subject based on the ideXlab platform.
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Sudden onset of disseminated Porokeratosis of Mibelli in a renal transplant patient.
Journal of the American Academy of Dermatology, 1999Co-Authors: K A Knoell, J W Patterson, B B WilsonAbstract:Porokeratosis is a disorder of epidermal keratinization of uncertain cause. Five clinical variants of Porokeratosis have been described. These include Porokeratosis of Mibelli, punctate Porokeratosis, linear Porokeratosis, Porokeratosis palmaris plantaris et disseminata, and disseminated superficial Porokeratosis. Disseminated superficial Porokeratosis and single plaque Porokeratosis of Mibelli have each been documented to occur in association with immunosuppression. To our knowledge, only 5 cases of disseminated Porokeratosis of Mibelli in transplant recipients have been reported. We present a patient who developed explosive onset of disseminated Porokeratosis of Mibelli shortly after renal transplantation. It is important to differentiate this unusual variety of Porokeratosis from other cutaneous manifestations in transplant patients so that appropriate therapy can be instituted.
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Sudden onset of disseminated Porokeratosis of Mibelli in a renal transplant patient
Journal of the American Academy of Dermatology, 1999Co-Authors: K A Knoell, J W Patterson, B B WilsonAbstract:Abstract Porokeratosis is a disorder of epidermal keratinization of uncertain cause. Five clinical variants of Porokeratosis have been described. These include Porokeratosis of Mibelli, punctate Porokeratosis, linear Porokeratosis, Porokeratosis palmaris plantaris et disseminata, and disseminated superficial Porokeratosis. Disseminated superficial Porokeratosis and single plaque Porokeratosis of Mibelli have each been documented to occur in association with immunosuppression. To our knowledge, only 5 cases of disseminated Porokeratosis of Mibelli in transplant recipients have been reported. We present a patient who developed explosive onset of disseminated Porokeratosis of Mibelli shortly after renal transplantation. It is important to differentiate this unusual variety of Porokeratosis from other cutaneous manifestations in transplant patients so that appropriate therapy can be instituted. (J Am Acad Dermatol 1999;41:830-2.)
K A Knoell - One of the best experts on this subject based on the ideXlab platform.
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Sudden onset of disseminated Porokeratosis of Mibelli in a renal transplant patient.
Journal of the American Academy of Dermatology, 1999Co-Authors: K A Knoell, J W Patterson, B B WilsonAbstract:Porokeratosis is a disorder of epidermal keratinization of uncertain cause. Five clinical variants of Porokeratosis have been described. These include Porokeratosis of Mibelli, punctate Porokeratosis, linear Porokeratosis, Porokeratosis palmaris plantaris et disseminata, and disseminated superficial Porokeratosis. Disseminated superficial Porokeratosis and single plaque Porokeratosis of Mibelli have each been documented to occur in association with immunosuppression. To our knowledge, only 5 cases of disseminated Porokeratosis of Mibelli in transplant recipients have been reported. We present a patient who developed explosive onset of disseminated Porokeratosis of Mibelli shortly after renal transplantation. It is important to differentiate this unusual variety of Porokeratosis from other cutaneous manifestations in transplant patients so that appropriate therapy can be instituted.
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Sudden onset of disseminated Porokeratosis of Mibelli in a renal transplant patient
Journal of the American Academy of Dermatology, 1999Co-Authors: K A Knoell, J W Patterson, B B WilsonAbstract:Abstract Porokeratosis is a disorder of epidermal keratinization of uncertain cause. Five clinical variants of Porokeratosis have been described. These include Porokeratosis of Mibelli, punctate Porokeratosis, linear Porokeratosis, Porokeratosis palmaris plantaris et disseminata, and disseminated superficial Porokeratosis. Disseminated superficial Porokeratosis and single plaque Porokeratosis of Mibelli have each been documented to occur in association with immunosuppression. To our knowledge, only 5 cases of disseminated Porokeratosis of Mibelli in transplant recipients have been reported. We present a patient who developed explosive onset of disseminated Porokeratosis of Mibelli shortly after renal transplantation. It is important to differentiate this unusual variety of Porokeratosis from other cutaneous manifestations in transplant patients so that appropriate therapy can be instituted. (J Am Acad Dermatol 1999;41:830-2.)
J W Patterson - One of the best experts on this subject based on the ideXlab platform.
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Sudden onset of disseminated Porokeratosis of Mibelli in a renal transplant patient.
Journal of the American Academy of Dermatology, 1999Co-Authors: K A Knoell, J W Patterson, B B WilsonAbstract:Porokeratosis is a disorder of epidermal keratinization of uncertain cause. Five clinical variants of Porokeratosis have been described. These include Porokeratosis of Mibelli, punctate Porokeratosis, linear Porokeratosis, Porokeratosis palmaris plantaris et disseminata, and disseminated superficial Porokeratosis. Disseminated superficial Porokeratosis and single plaque Porokeratosis of Mibelli have each been documented to occur in association with immunosuppression. To our knowledge, only 5 cases of disseminated Porokeratosis of Mibelli in transplant recipients have been reported. We present a patient who developed explosive onset of disseminated Porokeratosis of Mibelli shortly after renal transplantation. It is important to differentiate this unusual variety of Porokeratosis from other cutaneous manifestations in transplant patients so that appropriate therapy can be instituted.
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Sudden onset of disseminated Porokeratosis of Mibelli in a renal transplant patient
Journal of the American Academy of Dermatology, 1999Co-Authors: K A Knoell, J W Patterson, B B WilsonAbstract:Abstract Porokeratosis is a disorder of epidermal keratinization of uncertain cause. Five clinical variants of Porokeratosis have been described. These include Porokeratosis of Mibelli, punctate Porokeratosis, linear Porokeratosis, Porokeratosis palmaris plantaris et disseminata, and disseminated superficial Porokeratosis. Disseminated superficial Porokeratosis and single plaque Porokeratosis of Mibelli have each been documented to occur in association with immunosuppression. To our knowledge, only 5 cases of disseminated Porokeratosis of Mibelli in transplant recipients have been reported. We present a patient who developed explosive onset of disseminated Porokeratosis of Mibelli shortly after renal transplantation. It is important to differentiate this unusual variety of Porokeratosis from other cutaneous manifestations in transplant patients so that appropriate therapy can be instituted. (J Am Acad Dermatol 1999;41:830-2.)
Rudolf Happle - One of the best experts on this subject based on the ideXlab platform.
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Porokeratosis plantaris palmaris et disseminata caused by con genital pathogenic variants in the mvd gene and loss of hetero zygosity in affected skin
Acta Dermato-venereologica, 2021Co-Authors: Sabine Jagle, Hazem A Juratli, Geoffroy Hickman, Kira Sussmuth, Maria Del Carmen Boente, Julia Kopp, Peter Kirchmeier, Andreas Zimmer, Rudolf Happle, E BourratAbstract:Porokeratoses are a heterogeneous group of keratinization disorders. For linear Porokeratosis and disseminated superficial actinic Porokeratosis, a heterozygous pathogenic germline variant in a mevalonate pathway gene and a postzygotic second hit mutation present in affected skin have been shown to be the patho-genetic mechanism for the development of the lesions. However, the molecular mechanism leading to development of Porokeratosis plantaris, palmaris et disseminata is not known. This study analysed a cohort of 4 patients with linear Porokeratosis and 3 patients with Porokeratosis plantaris, palmaris et disseminata, and performed mutation analyses of DNA extracted from blood samples and skin biopsies. All of the study patients carried the heterozygous germline variant c.70+5G>A in the MVD gene. Loss of heterozygosity due to a second hit mutation was found in affected skin of 3 patients with linear Porokeratosis and 2 patients with Porokeratosis plantaris, palmaris et disseminata. These results suggest that Porokeratosis plantaris, palmaris et disseminata shares the same pathogenetic mechanism as other Porokeratosis subtypes and belongs to the phenotypic spectrum of MVD-associated Porokeratosis.
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Linear Porokeratosis superimposed on disseminated superficial actinic Porokeratosis: Report of two cases exemplifying the concept of type 1 segmental manifestation of autosomal dominant skin disorders
Journal of the American Academy of Dermatology, 1999Co-Authors: Pia Freyschmidt-paul, Rolf Hoffman, Arne König, Rudolf HappleAbstract:A concept of dichotomous types of segmental involvement of autosomal dominant skin disorders has recently been proposed. Among the different types of Porokeratosis, disseminated superficial actinic Porokeratosis is known to be an autosomal dominant skin disorder, and linear Porokeratosis represents the segmental form of the disease. We intended to exemplify the type 2 segmental manifestation within this concept. Clinical and histopathologic aspects of porokeratotic lesions of 2 patients were investigated. The family history was studied in both cases. Linear Porokeratosis superimposed on disseminated superficial actinic Porokeratosis was observed in both patients. These 2 cases of linear Porokeratosis associated with disseminated superficial actinic Porokeratosis can be taken as further examples of a type 2 segmental involvement occuring in an autosomal dominant skin disorder.
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cancer proneness of linear Porokeratosis may be explained by allelic loss
Dermatology, 1997Co-Authors: Rudolf HappleAbstract:Background: It is well known that Porokeratosis, a genetically heterogeneous disorder characterized by the histopathological feature of the cornoid lamella, shows an increased proneness to develop carcinoma. On the other hand, a significant mechanism in the origin of many forms of cancer is loss of heterozygosity or allelic loss. Objective: Because it has recently been proposed that linear Porokeratosis may result from allelic loss, one might expect that linear Porokeratosis is especially prone to malignant degeneration. In order to test this hypothesis, a review of case reports was performed. Method: Cases of cancer-associated Porokeratosis were collected from the European language literature and assigned to one of 5 different types [plaque type of Mibelli (PM); disseminated actinic superficial Porokeratosis (DSAP); Porokeratosis palmaris, plantaris et disseminata (PPPD); Porokeratosis punctata palmaris et plantaris (PPPP); linear Porokeratosis (LP)]. Results: Malignant or premalignant lesions were reported in 9 cases of PM, 15 cases of DSAP, 3 cases of PPPD, 1 case of PPPP and 21 cases of LP Conclusion: This analysis supports the view that among the various forms of Porokeratosis, the linear type is particularly susceptible to malignant degeneration. Arguments are presented in favor of the assumption that the genetic mechanism of allelic loss giving rise to LP may represent an initial step in the development of cancer.
Annalisa Patrizi - One of the best experts on this subject based on the ideXlab platform.
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Eruptive pruritic papular Porokeratosis in a Caucasian woman: a transient inflammatory stage of Porokeratosis.
Giornale italiano di dermatologia e venereologia : organo ufficiale Societa italiana di dermatologia e sifilografia, 2015Co-Authors: Annalisa Patrizi, Annalucia Virdi, Cosimo Misciali, Federico BardazziAbstract:Abstract Porokeratosis is usually asymptomatic, but a pruritic variant, called "eruptive pruritic papular Porokeratosis" or "inflammatory disseminated superficial Porokeratosis", has been described. We describe another case of pruritic Porokeratosis in a 63--year--old Caucasian woman with a poor response to antihistamines and local treatments. EPPP is uncommon in non--Asiatic people, but probably it is underestimated. We consider it a transient inflammatory stage rather than a distinct entity of Porokeratosis.
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Porokeratosis in the elderly: a new subtype of disseminated superficial actinic Porokeratosis.
Acta dermato-venereologica, 2000Co-Authors: Annalisa Patrizi, Beatrice Passarini, Carmine D'acunto, Iria NeriAbstract:In a review of all cases of Porokeratosis histologically diagnosed in our Department during the period 1991-98 we found that 12 patients (22%) were in their seventh to ninth decade. In all 12 (2 males and 10 females) the age of onset of the disease varied between 58 and 89 years (mean age 68.6 years). The clinical picture was similar in all the patients, with the number of lesions varying from a few to 20-50 annular plaques 10-15 mm in diameter, localized mainly on the lower limbs. We suggest that our patients had a very mild form of disseminated superficial actinic Porokeratosis confined to the extremities with an unusually late onset. This peculiar variety of late-onset disseminated superficial actinic Porokeratosis may represent a type of immunosuppression-induced Porokeratosis where the pathologic clone for Porokeratosis is present but remains latent until the amount of sun exposure, together with the physiological age-related lowering of immunocompetence, bring about its proliferation.
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Genital Porokeratosis of Mibelli.
Genitourinary medicine, 1995Co-Authors: Iria Neri, S. Marzaduri, Beatrice Passarini, Annalisa PatriziAbstract:Porokeratosis of Mibelli is a disorder of epidermal proliferation in which many different clinical forms can be distinguished. Two male patients with a localized type of Porokeratosis limited to the genitalia are reported. Later in life they developed an annular skin lesion with peripheral keratotic ridge. The histological examination of a biopsy specimen showed the characteristic features of Porokeratosis. There was no family history of similar skin disorders and the patients were not on any drugs. Genital Porokeratosis is probably underdiagnosed and we believe that these patients should be followed up on account of the precancerous potential of this disease.