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    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

    Treatment of disseminated superficial actinic porokeratosis with oral alitretinoin.
    J Eur Acad Dermatol Venereol 2017 May 22. Epub 2017 May 22.
    Department of Dermatology, College of Medicine, University of Hanyang, Seoul, Korea.
    Disseminated superficial actinic porokeratosis (DSAP), the most common type of porokeratosis, usually presents as large numbers of lesions on sun-exposed skin. Although several treatment options including corticosteroids, keratolytics, 5-fluorouracil, imiquimod, vitamin D3 analogues, cryotherapy, curettage, laser therapy and dermabrasion may be considered, there is no evidence-based standard treatment.(1-2) Patient 1 was a 26-year-old female who presented with a 2-year history of progressive scaly macules on both upper and lower extremities. 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 Mar 10. Epub 2017 Mar 10.
    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 Feb 27. Epub 2017 Feb 27.
    *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

    Somatic Mosaicism for a "Lethal" GJB2 Mutation Results in a Patterned Form of Spiny Hyperkeratosis without Eccrine Involvement.
    Pediatr Dermatol 2016 May 18;33(3):322-6. Epub 2016 Apr 18.
    Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
    Background: Spiny hyperkeratosis refers to a rare clinical phenotype characterized by nonfollicular keratotic projections and sometimes associated with other acquired and inherited conditions. We describe a case of congenital patterned spiny hyperkeratosis.

    Methods: To identify the cause of this disorder, we used a combination of whole exome sequencing, direct sequencing and TaqMan assay. 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 2017 Jun 23;108(5):e33-e37. Epub 2016 Mar 23.
    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

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