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    1073 results match your criteria Porokeratosis

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    Successful Use of Grenz Rays for Disseminated Superficial Actinic Porokeratosis: Report of 8 Cases.
    Dermatology 2017 Aug 18. Epub 2017 Aug 18.
    Department of Dermatology, University Hospital Zurich, Zurich, Switzerland.
    Background: Disseminated superficial actinic porokeratosis (DSAP) is a rare keratinization disorder with potential malignant transformation, for which present treatment strategies show limited success.

    Aim: To evaluate the response of DSAP lesions to grenz ray radiotherapy (RTx).

    Methods: Data of patients treated with RTx at University Hospital Zurich, Switzerland, between 2004 and 2015, were reviewed. Read More

    [Analysis of clinical and genetic features of nine patients with disseminated superfacial actinic porokeratosis].
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2017 Aug;34(4):481-485
    Shanghai Clinical College of Skin Disease, Anhui Medical University, Shanghai 200443, China.
    Objective: To analyze the clinical and genetic features of 9 ethnic Han Chinese patients with disseminated superfacial actinic porokeratosis (DSAP).

    Methods: Genomic DNA was extracted from peripheral blood samples collected from the patients. PCR and direct sequencing were carried out for five patients from a family, 4 sporadic cases, and 120 healthy controls to identify potential mutations of four genes (MVK, MVD, PMVK, FDPS) involved in the mevalonate pathway as well as SLC17A9, SSH1, and SART3 genes. Read More

    Disorder of the mevalonate pathway inhibits calcium-induced differentiation of keratinocytes.
    Mol Med Rep 2017 Aug 1. Epub 2017 Aug 1.
    Department of Dermatology, Institute of Dermatology, No. 1 Hospital, Anhui Medical University, Hefei, Anhui 230022, P.R. China.
    Mutation of genes encoding the enzymes of the mevalonate pathway cause a variety of diseases, including skin disorders. Mutation of four genes in this pathway, including mevalonate kinase, phosphomevalonate kinase, mevalonate diphosphate decarboxylase and farnesyl diphosphate synthase, have demonstrated to be responsible for porokeratosis (PK). However, the pathogenesis of PK remains unclear. Read More

    Follicular porokeratosis: four new cases.
    Clin Exp Dermatol 2017 Jul 27. Epub 2017 Jul 27.
    Department of Cellular Pathology, Warwick Hospital, Warwick, Warwickshire, UK.
    Porokeratosis, a disorder of keratinisation, is clinically characterized by the presence of annular plaques with a surrounding keratotic ridge. Clinical variants include linear, disseminated superficial actinic, verrucous/hypertrophic, disseminated eruptive, palmoplantar and porokeratosis of Mibelli (one or two typical plaques with atrophic centre and guttered keratotic rim). All of these subtypes share the histological feature of a cornoid lamella, characterized by a column of 'stacked' parakeratosis with focal absence of the granular layer, and dysmaturation (prematurely keratinised cells in the upper spinous layer). Read More

    Onychodystrophy due to porokeratosis of Mibelli: a rare association.
    Acta Dermatovenerol Alp Pannonica Adriat 2017 Jun;26(2):51-52
    Advanced Skin Clinic, Nashik, Maharashtra, India.
    Porokeratosis is a specific keratinization disorder that manifests clinically as well-demarcated annular or linear keratotic plaques of various sizes and forms and with distinguished histology showing cornoid lamella, which is a column of closely packed parakeratotic cells extending through the stratum corneum. Nail changes secondary to porokeratotic lesions involving digits are quite uncommon and rarely reported in the porokeratosis of Mibelli (PM) subtype. Here we report the case of a young girl with a PM lesion over the hand along with nail dystrophy of the involved digit. Read More

    Porokeratosis ptychotropica responding to photodynamic therapy: An alternative treatment for a refractory disease.
    Photodermatol Photoimmunol Photomed 2017 Jun 5. Epub 2017 Jun 5.
    Department of Dermatology, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain.
    Background: Porokeratosis ptychotropica (PP) is a rare variant of porokeratosis with a special predisposition to affect body folds, particularly the intergluteal cleft. This disease is resistant to most topical and systemic treatments, as shown in the review of the literature we provide here. Itching and discomfort are often a difficult problem to solve. Read More

    Disseminated superficial actinic porokeratosis treated with ingenol mebutate gel 0.05.
    Cutis 2017 Mar;99(3):E36-E39
    Department of Dermatology, Icahn School of Medicine at Mount Sinai, New York, New York, USA.
    Disseminated superficial actinic porokeratosis (DSAP) is a chronic condition characterized by numerous atrophic papules and patches with a distinctive peripheral keratotic ridge, typically found on sun-exposed areas. Treatment of DSAP is warranted not only for cosmetic and symptomatic benefits but also to prevent malignant transformation. Successful treatment of DSAP often is difficult and frequently requires the use of multiple modalities. Read More

    Porokeratosis ptychotropica.
    An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):134-136
    Universidade de Santo Amaro (UNISA) - Santo Amaro (SP), Brazil.
    Porokeratosis is a skin disorder clinically characterized by annular plaques with keratotic borders resembling the Great Wall of China and histopathologically by cornoid lamellae. The disease has several clinical variants. Porokeratosis ptychotropica, which has recently become part of these variants, is quite rare and little known. Read More

    Porokeratosis of Mibelli in an HIV-positive patient.
    An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):131-133
    Hospital do Servidor Público Estadual de São Paulo (HSPE) - São Paulo (SP) - Brazil.
    Porokeratosis represents a group of disorders of epidermal keratinization that are characterized by one or more annular plaques surrounded by a histologically distinctive hyperkeratotic ridge-like border called the cornoid lamella. Many studies showed that organ transplantation and immunosuppression were associated in a significant number of cases. Furthermore, an association with squamous cell carcinoma and basal cell carcinoma has been noted in all variants of porokeratosis. Read More

    Porokeratosis simulating Bowen's disease on dermoscopy.
    An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):119-121
    Universidade de Pernambuco (UPE) - Recife (PE), Brazil.
    Porokeratosis is a disorder of epidermal keratinization characterized by the presence of annular hyperkeratotic plaques. Its etiopathogenesis is not yet fully understood, but a relationship with immunosuppression has been reported. Dermoscopic examination revealed a classic yellowish-white ring-like structure that resembled "volcanic crater contour" - the so-called cornoid lamella. Read More

    Treatment of Porokeratosis: A Systematic Review.
    Am J Clin Dermatol 2017 Aug;18(4):435-449
    Department of Dermatology, University Hospital Jena, Erfurter Straße 35, 07743, Jena, Germany.
    Background: Porokeratosis (PK) is a rare skin disease of unknown etiology. It consists of a keratinization disorder, which may appear in several clinical forms and can undergo malignant transformation. The histopathological hallmark of PK is the cornoid lamella. Read More

    Follicular Porokeratosis, a Porokeratosis Variant.
    Am J Dermatopathol 2017 Aug;39(8):e107-e109
    *Department of Dermatopathology, Pathlab Bay of Plenty, Tauranga, New Zealand; †Skin Dermatology Institute, Tauranga, New Zealand; and ‡Department of Pathology and Molecular Medicine, University of Auckland.
    Porokeratosis derives from a process of abnormal keratinization, resulting in clinical and histologic variants. Follicular involvement is infrequently described, with previous suggestions that it may represent a distinct condition. We describe a case of typical disseminated superficial actinic porokeratosis with additional clinically prominent folliculocentric keratosis. Read More

    Light and laser treatment modalities for disseminated superficial actinic porokeratosis: a systematic review.
    Lasers Med Sci 2017 May 27;32(4):945-952. Epub 2017 Feb 27.
    Division of Dermatology, Creighton University School of Medicine, Omaha, NE, USA.
    Treatment of disseminated superficial actinic porokeratosis (DSAP) is poorly standardized. The present review seeks to comprehensively discuss the potential for laser and light modalities in the treatment of DSAP. A systematic review of light and laser treatment modalities was conducted to include 26 cases of patients with DSAP. Read More

    Secondary Cutaneous Amyloidosis in a Patient with Mycosis Fungoides.
    Ann Dermatol 2017 Feb 3;29(1):79-82. Epub 2017 Feb 3.
    Department of Dermatology, Dankook University Medical College, Cheonan, Korea.
    Secondary cutaneous amyloidosis refers to clinically unapparent amyloid deposits within the skin in association with a pre-existing skin condition or skin tumors, such as basal cell carcinoma, porokeratosis, solar elastosis, Bowen's disease, and mycosis fungoides. A 70-year-old woman presented with a 6-month history of asymptomatic multiple yellowish plaques on both legs. She had been diagnosed with mycosis fungoides 7 years ago and was treated with psoralen and ultraviolet A radiation (PUVA) therapy, narrow-band ultraviolet B (UVB) therapy, and acitretin for 5 years. Read More

    Craniosynostosis, delayed closure of the fontanelle, anal, genitourinary, and skin abnormalities (CDAGS syndrome): first report in a Mexican patient and review of the literature.
    Int J Dermatol 2017 Apr 19;56(4):435-439. Epub 2017 Feb 19.
    Department of Dermatology, Hospital Infantil de México Federico Gómez, México, D.F, Mexico.
    Introduction: Craniosynostosis and clavicular hypoplasia, delayed closure of the fontanelle, cranial defects, anal and genitourinary abnormalities, and skin (CDAGS), is an infrequent autosomal recessive entity with only 10 cases reported; no associated gene has been identified so far.

    Case Report: The proband is a 2-year-old Mexican female with brachycephaly, cleft palate, anal malformation with rectovestibular fistula, and clinodactyly of the third toe overlapping the second. At 4 months of age, she developed a disseminated dermatosis with erythematous scaly nummular plaques, elevated keratotic sharp borders with thin to broad flaking, hematic crusts, and keratotic surface in others. Read More

    Successful management of disseminated superficial actinic porokeratosis with diclofenac sodium 3% gel.
    Dermatol Ther 2017 May 18;30(3). Epub 2016 Nov 18.
    Dermatology Department, Hospital Universitario Lucus Augusti, Lugo. C /Ulises Romero no. 1 CP:, 27003, Lugo, Galicia, Spain.

    [Circumscribed palmar hypokeratosis (CPM): The diagnostic value of dermoscopy].
    Ann Dermatol Venereol 2017 Mar 19;144(3):197-202. Epub 2016 Oct 19.
    Service de dermatologie, CHU d'Angers, 4, rue Larrey, 49933 Angers cedex 9, France. Electronic address:
    Background: Circumscribed palmar hypokeratosis (CPH) is a rare skin disease, first described in 2002, associated with sudden localized reduction of the corneal layer. In most cases, it presents as an isolated rounded erythematous palmar lesion on the thenar eminence. We describe the dermoscopic semiology of CPH in 3 cases. Read More

    Mycosis fungoides patient accompanied actinic keratosis, actinic keratosis with squamous cell carcinoma transformation, and porokeratosis after NBUVB therapy - 1st case report and review of the literature.
    Medicine (Baltimore) 2016 Oct;95(41):e5043
    aDepartment of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology bDepartment of Dermatology, The 457th Airforce Hospital, Wuhan cDepartment of Dermatology, Huizhou Central Hospital, Guangdong, P.R. China.
    Introduction: Mycosis fungoides (MF) is the most common form of primary cutaneous T cell lymphoma. Narrowband ultraviolet B light (NBUVB) is used increasingly in treating MF because of its good toleration and well-established management.

    Concerns: To discuss the risk factors and underlying pathogenic factors in the patients with secondary skin diseases after NBUVB therapy. Read More

    Dermoscopy in General Dermatology: A Practical Overview.
    Dermatol Ther (Heidelb) 2016 Dec 9;6(4):471-507. Epub 2016 Sep 9.
    Department of Experimental and Clinical Medicine, Institute of Dermatology, University of Udine, Udine, Italy.
    Over the last few years, dermoscopy has been shown to be a useful tool in assisting the noninvasive diagnosis of various general dermatological disorders. In this article, we sought to provide an up-to-date practical overview on the use of dermoscopy in general dermatology by analysing the dermoscopic differential diagnosis of relatively common dermatological disorders grouped according to their clinical presentation, i.e. Read More

    Porokeratosis ptychotropica: a rare manifestation with typical histological exam.
    An Bras Dermatol 2016 Jul-Aug;91(4):496-8
    Santa Casa de Misericórdia de São Paulo - São Paulo (SP), Brazil.
    Porokeratosis is a disorder of epidermal keratinization characterized clinically by a distinctive ridge-like border, and histologically by cornoid lamellae. The known clinical variants of porokeratosis are: classic porokeratosis of Mibelli, disseminated superficial (actinic) porokeratosis (DSAP), porokeratosis palmaris et plantaris disseminata, linear porokeratosis and punctate porokeratosis. In 1995, a seventh form was described as porokeratosis ptychotropica: a verrucous form resembling psoriasis involving the gluteal cleft presenting on the histological exam multiple cornoid lamellae. Read More

    Clinical and dermatoscopic features of porokeratosis palmaris et plantaris.
    Indian Dermatol Online J 2016 Jul-Aug;7(4):290-2
    Department of Dermatology, Mahatma Gandhi Mission Medical College and Hospital, Navi Mumbai, Mumbai, Maharashtra, India.
    A dermatoscope is an important tool in a dermatologist's armamentarium as it can eliminate the need for a biopsy in a wide array of conditions. Porokeratosis was described by Mibelli and Respighi in 1893, as a disorder of keratinization which on the basis of distribution patterns was described as five clinical variants that portrayed a coronoid lamella on histopathology. We describe a case of asymptomatic, long-standing palmar and plantar pits, which on dermatoscopy showed features suggestive of porokeratosis, which was later reconfirmed by histopathologic sections. Read More

    Disseminated superficial actinic porokeratosis (DSAP): significant improvement after local administration of calcipotriol/betamethasone gel?
    Wien Med Wochenschr 2017 Mar 28;167(3-4):85-88. Epub 2016 Jul 28.
    Medical Institute of Minitry of Interior (MVR-Sofia), Chair of Department of Dermatology, Venereology and Dermatologic Surgery, General Skobelev Nr 79, 1606, Sofia, Bulgaria.
    Porokeratosis is defined as a disorder of keratinization characterized by one or more atrophic patches surrounded by a clinically and histologically distinctive hyperkeratotic ridgelike border called the cornoid lamella. Lesions are most commonly located on the sun-exposed sides of trunk and extremities, while exclusive facial involvement has been also reported. Despite that the exact risk of cutaneous malignancy developing in porokeratosis is unknown, it is confirmed by series of case reports, including squamous cell carcinoma, basal cell carcinoma and even melanoma. Read More

    Mutations in the mevalonate pathway genes in Chinese patients with porokeratosis.
    J Eur Acad Dermatol Venereol 2016 Sep 16;30(9):1512-7. Epub 2016 Jul 16.
    Department of Dermatology, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.
    Background: Porokeratosis (PK, MIM 175800) is a chronic autosomal dominant cutaneous keratinization disorder, which has a wide variety of clinical manifestations.

    Objectives: We analysed the molecular basis of 10 families and 12 sporadic cases with different subtypes of porokeratosis in the Chinese population.

    Methods: Genomic DNA was extracted from peripheral blood samples. Read More

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