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    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 Feb 19. 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

    Wood's lamp image of porokeratosis.
    Photodermatol Photoimmunol Photomed 2016 Dec 16. Epub 2016 Dec 16.
    Department of Dermatology and Venereology, Xuanwu hospital, Capital Medical University.
    Porokeratosis (PK), initially described by Mibelli in 1893, is a skin disorder of epidermal keratinization. This disorder is clinically characterized by annular plaques with edges and atrophic centers, as well as histopathologically characterized by the presence of cornoid lamella. The etiology of this disease remains unknown. Read More

    Successful management of disseminated superficial actinic porokeratosis with diclofenac sodium 3% gel.
    Dermatol Ther 2016 Nov 18. 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 2016 Oct 19. 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

    Linear porokeratosis with multiple squamous cell carcinomas successfully treated by electrochemotherapy.
    Br J Dermatol 2016 Dec 17;175(6):1342-1345. Epub 2016 Aug 17.
    Department of Dermatology, Royal London Hospital, Whitechapel, London, U.K.
    Porokeratosis is a clonal epidermal disorder of keratinization characterized by annular lesions with an atrophic centre and a hyperkeratotic edge. The cornoid lamella is the histopathological hallmark. Six clinical variants are recognized: porokeratosis of Mibelli; disseminated superficial porokeratosis; disseminated superficial actinic porokeratosis (DSAP); porokeratosis plantaris et palmaris disseminata; punctate porokeratosis and linear porokeratosis. Read More

    Loss-of-function Mutation in PMVK Causes Autosomal Dominant Disseminated Superficial Porokeratosis.
    Sci Rep 2016 Apr 7;6:24226. Epub 2016 Apr 7.
    Key Laboratory of Molecular Biophysics of Ministry of Education, Department of Genetics and Developmental Biology, College of Life Science and Technology, Huazhong University of Science and Technology, Wuhan, Hubei 430074, PR China.
    Disseminated superficial porokeratosis (DSP) is a rare keratinization disorder of the epidermis. It is characterized by keratotic lesions with an atrophic center encircled by a prominent peripheral ridge. We investigated the genetic basis of DSP in two five-generation Chinese families with members diagnosed with DSP. Read More

    Eruptive Disseminated Porokeratosis in a Patient With Type 2 Diabetes Mellitus.
    Am J Dermatopathol 2016 Aug;38(8):e125-7
    *Department of Dermatology, Yildirim Beyazit University, Ankara Numune Educational and Research Hospital, Ankara, Turkey; and Departments of †Dermatology, and ‡Pathology, Ankara Numune Educational and Research Hospital, Ankara, Turkey.
    Porokeratosis includes a group of heterogenous disorders that represents distinct clinical subtypes of the same genetic pattern. Although entire pathogenesis of porokeratosis still remains unknown, certain factors including ultraviolet radiation and immunosuppression are suggested to be some of the factors inducing this disorder. Eruptive disseminated porokeratosis is a recently described form of porokeratosis, which frequently occurs in the presence of immunosuppresion or malignancy. Read More

    Diagnosing porokeratosis of Mibelli every time: a novel biopsy technique to maximize histopathologic confirmation.
    Cutis 2016 Mar;97(3):188-90
    University of Mississippi Medical Center, Jackson and School of Medicine and Dentistry, University of Rochester Medical Center, New York, USA.
    Porokeratosis of Mibelli (PM) is a rare condition with the potential for malignant transformation that presents a clinical and pathologic diagnostic challenge. An improperly oriented biopsy may lead to the wrong histopathologic diagnosis. We report a case of PM that was previously misdiagnosed and describe a biopsy technique for suspected PM that maximizes the potential for histopathologic confirmation of the diagnosis. Read More

    Dermoscopy of Disseminated Superficial Actinic Porokeratosis.
    Actas Dermosifiliogr 2016 Mar 22. Epub 2016 Mar 22.
    Cátedra de Dermatología "Prof. Miguel Martínez", Hospital de Clínicas "Dr. Manuel Quintela", Montevideo, Uruguay.
    We present a series of 6 cases of disseminated superficial actinic porokeratosis and describe their dermoscopic features. Dermoscopy is a noninvasive in vivo technique that is useful as a tool for the diagnosis and follow-up of porokeratosis. This condition has specific dermoscopic features that were observed in our series of cases and that are consistent with reports in the international literature. Read More

    Eruptive angiokeratomas and porokeratosis in the setting of sclerodermatous graft-vs.-host disease.
    J Cutan Pathol 2016 Jun 15;43(6):516-519. Epub 2016 Apr 15.
    Department of Pathology, Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, NY, USA.
    Porokeratosis and angiokeratomas are both seen in the setting of chronic graft-vs.-host disease (GVHD), but rarely occur together. We present a case of a patient with lichen planus-like (LPL) and sclerodermatous chronic GVHD manifesting after allogeneic bone marrow transplant with concomitant eruptive angiokeratomas and porokeratosis. Read More

    Pigmented Porokeratosis. A Further Variant?
    Am J Dermatopathol 2016 Mar;38(3):218-21
    *Pathlab Tauranga, Tauranga, New Zealand; and †DaVinci Clinic, Tauranga, New Zealand.
    Porokeratosis is a clonal disorder of keratinization characterized by the presence of the cornoid lamella. A number of variants of porokeratosis have been described, based on the clinical features and histologic features of the lesions. The authors present a case of porokeratosis with prominent melanocytic hyperplasia, which was biopsied to clinically exclude melanoma. Read More

    Photoletter to the editor: Response of linear porokeratosis to photodynamic therapy in an 11-year-old girl.
    J Dermatol Case Rep 2015 Dec 31;9(4):118-9. Epub 2015 Dec 31.
    Department of Dermatology, Virgen de las Nieves Hospital, Granada, Spain;
    Porokeratoses are a group of different entities that belong to the skin keratinization disorders. From the histological point of view the main and common characteristic of these disorders is the presence of compact parakeratotic columns known as cornoid lamellae. All varieties should be carefully treated and followed-up because of the risk of developing malignant epithelial tumors. Read More

    Disseminated superficial actinic porokeratosis improved with fractional 1927-nm laser treatments.
    J Cosmet Laser Ther 2016 28;18(1):53-5. Epub 2016 Jan 28.
    d Department of Dermatology , Brown Medical School , Providence , RI , USA.
    Disseminated superficial actinic porokeratosis (DSAP) is an inherited disorder of keratinization readily diagnosed through clinical and histologic examination. While generally benign in nature, the lesions can have profound psychosocial implications for patients. Although no cure exists, a number of treatment modalities, from topical medications to laser and light devices, have been reported with variable success. Read More

    Identification of three mutations in the MVK gene in six patients associated with disseminated superficial actinic porokeratosis.
    Clin Chim Acta 2016 Feb 12;454:124-9. Epub 2016 Jan 12.
    Key Laboratory of Molecular Biophysics of Ministry of Education, Department of Genetics and Developmental Biology, College of Life Science and Technology, Huazhong University of Science and Technology, Wuhan, Hubei 430074, PR China. Electronic address:
    Porokeratosis is recognized as a heterogenously inherited epidermal keratinization disorder. Disseminated superficial actinic porokeratosis (DSAP) is considered to be the most common form of porokeratosis and is characterized by multiple, small keratotic lesions on sun-exposed areas of body. MVK has been reported to be the main candidate gene associated with DSAP. Read More

    Hyperkeratotic variant of porokeratosis in a patient with Hepatitis C virus infection and a concomitant immunosuppressed state.
    Dermatol Online J 2015 Nov 18;21(11). Epub 2015 Nov 18.
    University of Alabama at Birmingham.
    Porokeratoses are acquired and hereditary disorders of keratinization that share a distinctive lesion characterized by raised keratotic borders corresponding histologically to an angled column of parakeratotic cells, called a cornoid lamella. Although a precise mechanistic explanation is lacking, ultraviolet radiation and immunosuppressed states are considered causally-associated with most cases of acquired porokeratosis. Hepatitis C virus (HCV) infection has been proposed as a link between the immunosuppressed states and development of acquired porokeratosis. Read More

    Premalignant and Malignant Skin Lesions in Two Recipients of Vascularized Composite Tissue Allografts (Face, Hands).
    Case Rep Transplant 2015 13;2015:356459. Epub 2015 Oct 13.
    Department of Transplantation, Edouard Herriot Hospital Group, 69003 Lyon, France.
    Recipients of solid organ transplants (RSOT) have a highly increased risk for developing cutaneous premalignant and malignant lesions, favored by the lifelong immunosuppression. Vascularized composite tissue allografts (VCA) have been introduced recently, and relevant data are sparse. Two patients with skin cancers (one with basal cell carcinoma and one with squamous cell carcinomas) have been so far reported in this patient group. Read More

    [Verrucous variant of porokeratosis of Mibelli as a differential diagnosis of psoriasis vulgaris].
    Hautarzt 2016 Mar;67(3):244-8
    Klinik für Dermatologie, Allergologie und Phlebologie, Klinikum Bremerhaven Reinkenheide, Bremerhaven, Postbrookstr. 103, 27574, Bremerhaven, Deutschland.
    In a 37-year-old man, diagnosis of verrucous porokeratosis could only be made by histological examination. Previously, the skin lesions on the right buttock had been treated by several dermatologists as psoriasis vulgaris. The clinical picture of both dermatoses was characterized by sharply defined, erythematous papules and plaques. Read More

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