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    721 results match your criteria Pityriasis Rubra Pilaris

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    Efficacy of Triaging Direct Immunofluorescence in Intraepidermal Bullous Dermatoses.
    Am J Dermatopathol 2017 Mar 24. Epub 2017 Mar 24.
    *Department of Pathology, University of South Dakota-Sanford School of Medicine, Sioux Falls, SD; and †Departments of Pathology and Dermatology, Cleveland Clinic Foundation, Cleveland, OH.
    Background: Direct immunofluorescence (DIF) is considered pivotal in diagnosing autoimmune blistering diseases. Our goal was to examine the necessity of DIF in intraepidermal bullous cases, of which pemphigus vulgaris (PV) is the prototype.

    Methods: Sixty-six cases from 2010 to 2014 submitted for DIF with an intraepidermal blistering disease listed in the differential diagnosis were reviewed by 2 board-certified dermatopathologists to see if they would order DIF based on routine histologic findings. Read More

    Beneficial effect of ustekinumab in familial pityriasis rubra pilaris with a new missense mutation in CARD14.
    Br J Dermatol 2017 Mar 16. Epub 2017 Mar 16.
    St John's Institute of Dermatology, King's College London (Guy's Campus), London, UK.
    Pityriasis rubra pilaris (PRP) represents a group of rare chronic inflammatory skin disorders in which ~1 in 20 affected individuals show autosomal dominant inheritance. In such cases, there may be gain-of-function mutations in CARD14, encoding caspase recruitment domain-containing protein 14 (CARD14) that activates the non-canonical nuclear factor-kappa B (NF-κB) pathway, thereby promoting cutaneous inflammation. Here, we report a mother and son with PRP due to a new missense mutation in CARD14 and describe the beneficial clinical effects of ustekinumab, a monoclonal antibody against interleukins-12 and -23, in both subjects. Read More

    Autosomal dominant familial generalized pustular psoriasis caused by a CARD14 mutation.
    Br J Dermatol 2017 Mar 10. Epub 2017 Mar 10.
    Department of Dermatology, Shinshu University, Graduate School of Medicine, Matsumoto, Japan.
    In 2012, gain-of-function mutations in CARD14, which encodes caspase recruitment domain family member 14, were identified as the cause of familial psoriasis vulgaris (PV) and familial pityriasis rubra pilaris (PRP).(1,2) We and another group reported that CARD14 variants are associated with generalized pustular psoriasis (GPP) and palmoplantar pustular psoriasis (PPP).(3-5) The other reports mentioned that CARD14 mutations in individuals with GPP and erythrodermic PRP. Read More

    Differentiation of pityriasis rubra pilaris from plaque psoriasis by dermoscopy.
    Arch Dermatol Res 2017 May 9;309(4):311-314. Epub 2017 Mar 9.
    Department of Dermatology, Venereology and Andrology, Faculty of Medicine, Assiut University, Assiut, Egypt.
    Pityriasis rubra pilaris (PRP) and plaque psoriasis (PP) are two distinctive erythemato-squamous skin diseases that often have to be differentiated from each other and from other similar dermatoses. Dermoscopy has been proven to aid the clinical diagnosis of several inflammatory disorders, minimizing the need for skin biopsy. Our aim was to determine the dermoscopic patterns of PRP compared to PP and to assess the significance of certain dermoscopic criteria in the diagnosis of PRP. Read More

    Interleukin 23-Helper T Cell 17 Axis as a Treatment Target for Pityriasis Rubra Pilaris.
    JAMA Dermatol 2017 Apr;153(4):304-308
    Department of Dermatology and Venereology, University Hospital of Lausanne, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
    Importance: Treatment of pityriasis rubra pilaris (PRP) is solely based on its resemblance to psoriasis rather than any knowledge of its pathomechanism. Insight into pathogenic mediators of inflammation is essential for targeted and valid treatment options that could replace previous serendipitous therapeutic approaches in refractory PRP.

    Objective: To determine whether blockade of the interleukin 23-helper T cell 17 (IL-23-TH17) pathway with ustekinumab represents an efficacious and, based on its proinflammatory cytokine profile, targeted treatment option in PRP. Read More

    Psoriasiform dermatitides: A brief review.
    Semin Diagn Pathol 2017 May 14;34(3):220-225. Epub 2016 Dec 14.
    Division of Surgical Pathology & Cytopathology, University of Virginia Health System, Room 3020, University of Virginia Hospital, 1215 Lee Street, Charlottesville, VA 22908-0214, United States. Electronic address:
    Psoriasis vulgaris (PV)-the prototypical "psoriasiform" dermatitis-may assume a spectrum of histologic appearances, depending on whether it has been treated or not. Because of that relative lack of morphological uniformity, other skin disorders that feature epidermal acanthosis, with or without associated inflammation, may be confused diagnostically with PV. This brief review considers the clinicopathologic attributes of PV and its imitators, including chronic spongiotic dermatitides, lichen simplex chronicus, prurigo nodularis, pityriasis rubra pilaris, psoriasiform secondary syphilis, and Reiter syndrome. Read More

    Paraneoplastic pityriasis rubra pilaris: case report and literature review.
    Clin Exp Dermatol 2017 Jan 29;42(1):54-57. Epub 2016 Nov 29.
    Department of Dermatology, Tel Aviv Sourasky Medical Centre, Tel Aviv, Israel.
    Pityriasis rubra pilaris (PRP; MIM 173200) is an uncommon papulosquamous inflammatory dermatosis. Only a few cases of PRP associated with an underlying malignancy have been documented. We investigated a 59-year-old patient presenting with a fulminant form of PRP recalcitrant to systemic retinoid therapy, in whom the skin disease heralded a diagnosis of cholangiocarcinoma. Read More

    Follicular Psoriasis: Differentiation from Pityriasis Rubra Pilaris-An Illustrative Case and Review of the Literature.
    Pediatr Dermatol 2017 Jan 22;34(1):e65-e68. Epub 2016 Nov 22.
    Department of Dermatology, University of Minnesota, Minneapolis, Minnesota.
    The follicular presentation of psoriasis is a well-described but uncommon variant. In some cases, follicular psoriasis may clinically and histopathologically mimic pityriasis rubra pilaris. There are several reports discussing the resemblance of widespread follicular psoriasis in children to pityriasis rubra pilaris. Read More

    Pityriasis rubra pilaris-like eruption following insulin therapy initiation.
    Dermatol Pract Concept 2016 Oct 31;6(4):19-21. Epub 2016 Oct 31.
    National Center of Pharmacovigilance, Faculty of Medicine, University of Tunis El Manar, Tunisia.
    Pityriasis rubra pilaris (PRP) is a chronic disorder of keratinization of unclear pathogenesis. PRP-like eruptions induced by drugs have rarely been described. A previously healthy 29-year-old man presented with a generalized, rapidly spreading, erythematosquamous dermatosis, that started three days after initiation of subcutaneous insulin therapy for diabetes mellitus type 1. Read More

    Pityriasis Rubra Pilaris Type V as an Autoinflammatory Disease by CARD14 Mutations.
    JAMA Dermatol 2017 Jan;153(1):66-70
    Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
    Importance: We found CARD14 mutations (2 de novo novel mutations and another previously reported mutation) in 3 of 3 patients with pityriasis rubra pilaris (PRP) type V, but not in patients with PRP of other types. Our findings, combined with the published literature, suggest that type V PRP, both familial and sporadic, can be caused by CARD14 mutations. Detailed clinical observation revealed that all 3 patients displayed unique patchy macular brown hyperpigmentation. 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

    Clinicopathological Study of Non-Infectious Erythaematous Papulosquamous Skin Diseases.
    J Clin Diagn Res 2016 Jun 1;10(6):EC19-22. Epub 2016 Jun 1.
    Professor and Head, Department of Pathology, A J Institute of Medical Sciences , Kuntikana, Mangalore, Karnataka, India .
    Introduction: Papulosquamous diseases are characterized by scaly papules and plaques with similar clinical picture which amounts to confusion and hence, a definitive histopathological diagnosis goes a long way in treatment of such diseases.

    Aim: The aim of the study was to study the histomorphology of non-infectious, erythaematous, papulosquamous lesions of skin with clinicopathological correlation.

    Materials And Methods: Skin biopsies from 150 clinically diagnosed/suspected non-infectious erythaematous, papulosquamous skin diseases were received in the Department of Pathology. Read More

    Bevacizumab-induced pityriasis rubra pilaris-like eruption.
    Proc (Bayl Univ Med Cent) 2016 Jul;29(3):335-6
    Department of Dermatology, Baylor Scott & White Health/Texas A&M Health Science Center College of Medicine, Temple, Texas.
    Pityriasis rubra pilaris is a rare inflammatory disorder characterized by follicular papules on an erythematous base often exhibiting islands of unaffected skin, follicular plugging, and palmoplantar hyperkeratosis. While vitamin A deficiency and autoimmune reactions have been hypothesized as possible etiologies of this condition, pityriasis rubra pilaris-like eruptions secondary to medications are extremely rare. To our knowledge, only three other cases have been reported, and pityriasis rubra pilaris has never been reported in association with bevacizumab. Read More

    [Off-label indications of biological drugs in dermatology].
    Rev Med Suisse 2016 Mar;12(512):635-8
    Last years, the development of biological drugs substantially improved the outcome of many inflammatory diseases. In dermatology, this kind of therapy is essentially prescribed for the treatment of psoriasis, and include the anti-TNF, anti-IL-12/IL-23 and anti-IL-17. Despite these treatments have not yet been approved, they seemed to be efficient for the treatment of many other inflammatory dermatosis, like granulomatous diseases, neutrophilic diseases, hydradenitis suppurativa or pityriasis rubra pilaris. Read More

    Wong-type dermatomyositis: a mimic of many dermatoses.
    J Cutan Pathol 2016 Sep 13;43(9):781-6. Epub 2016 Jun 13.
    Department of Dermatology, University of Cincinnati, Cincinnati, OH, USA.
    Wong-type dermatomyositis (DM) is a rare variant characterized by keratotic follicular papules that may mimic pityriasis rubra pilaris. Histopathologic examination shows follicular and non-follicular epidermal invaginations filled with keratin. The diagnosis is often delayed. Read More

    Late onset pityriasis rubra pilaris type IV treated with low-dose acitretin.
    Acta Dermatovenerol Alp Pannonica Adriat 2016 ;25(1):15-7
    Department of Dermatology, Porto Central Hospital, Porto, Portugal.
    Pityriasis rubra pilaris is a chronic inflammatory dermatosis of unknown etiology and great clinical variability. It has been divided into six categories. Types III, IV, and V occur in childhood and are distinguished by their clinical presentation, age of onset, and course. Read More

    Epidemiologic, Clinicopathologic, Diagnostic, and Management Challenges of Pityriasis Rubra Pilaris: A Case Series of 100 Patients.
    JAMA Dermatol 2016 Jun;152(6):670-5
    Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennsylvania.
    Importance: Pityriasis rubra pilaris (PRP) is a rare papulosquamous disorder with limited epidemiologic and clinicopathologic data. Little information is available on long-term outcomes, comorbidities, and treatment efficacy.

    Objective: To evaluate objective and subjective disease experience metrics from the perspectives of patients and clinicians. Read More

    Pityriasis Rubra Pilaris in a 3-Year-Old Male.
    Mil Med 2016 Mar;181(3):e298-301
    Department of Clinical Specialties and Dermatology Services, U.S. Army Health Center-Vicenza, CMR 427, APO AE 09630.
    A 3-year-old male presented with progressive pruritic red-orange plaques across most of his body with erythema, desquamation, and fissuring of the hands and feet. He was diagnosed with classic juvenile (type III) pityriasis rubra pilaris (PRP) and treated with oral isotretinoin for 6 months. His skin findings resolved quickly during the treatment period, with residual postinflammatory hypopigmentation resolving within a year. Read More

    [Erythematosquamous dermatoses in adolescence].
    Hautarzt 2016 Apr;67(4):293-7
    Klinik für Dermatologie, Venerologie und Allergologie, Kinderdermatologische Ambulanz, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland.
    Erythematosquamous dermatoses in adolescents comprise a wide range of differential diagnoses. Age-typical variations of the clinical manifestation, the need to differentiate common conditions from rare diseases as well as the tremendous psychosocial impact which the patients perceive especially in this vulnerable period of life can become major challenges for pediatric dermatologists. This article summarizes key features of common erythematosquamous dermatoses and less frequent skin diseases occurring during adolescence. Read More

    Onychogryphosis: A Report of Ten Cases.
    Skinmed 2015 1;13(5):355-9. Epub 2015 Oct 1.
    Medical Student on Elective Dermatology Rotation, Guatemala City, Guatemala.
    Many diseases can result in nail thickening, such as psoriasis, onychomycosis, pityriasis rubra pilaris, pachyonychia, and ichthyosis. In this report, the authors discuss such a condition, called onychogryphosis. Onychogryphosis is a thickening of the nail plate, with associated gross hyperkeratosis and increased curvature. Read More

    Severe, Disfiguring, Pityriasis Rubra Pilaris in a Woman in the Dominican Republic: Histopathologic Diagnosis and Response to Antiretroviral Therapy.
    J Int Assoc Provid AIDS Care 2016 Jan-Feb;15(1):11-4. Epub 2015 Oct 29.
    Clínica de Familia La Romana, La Romana, Dominican Republic Department of Epidemiology and Public Health, Miller School of Medicine, University of Miami, Miami, FL, USA.
    Pityriasis rubra pilaris (PRP) is a poorly understood dermatologic condition usually accompanied by keratoderma and intense erythroderma with islands of unaffected skin. The PRP categories include HIV-associated PRP VI. A 23-year-old HIV-infected, dark-skinned woman in the Dominican Republic developed an extremely severe, disfiguring process characterized first by a dry scaly rash involving her face, trunk, and extremities with hyperpigmentation and islands of spared skin and minimal erythroderma, followed by alopecia and development of a thick horny layer on the scalp and face. Read More

    Sofosbuvir-Induced Erythrodermic Pityriasis Rubra Pilaris-Like Drug Eruption.
    J Drugs Dermatol 2015 Oct;14(10):1161-2
    Until 2011, the standard-of-care therapy for patients with hepatitis C consisted of interferon and ribavirin. The recent advent of new targeted therapies against this virus has provided more options of treatment for infected patients. Sofosbuvir, a nucleotide inhibitor of hepatitis C virus (HCV) RNA polymerase, was recently approved by the US Food and Drug Administration in 2013. Read More

    The Incidence of Acantholysis in Pityriasis Rubra Pilaris-Histopathological Study Using Multiple-Step Sections and Clinicopathologic Correlations.
    Am J Dermatopathol 2015 Oct;37(10):755-8
    *Department of Dermatology, Rambam Health Care Campus, Haifa, Israel; and †The Ruth and Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel.
    Background: The classical histopathological findings in the epidermis of pityriasis rubra pilaris (PRP) do not include acantholysis; however, acantholysis was described in several case reports and a few series of PRP with variable frequencies. We sought to establish the incidence of acantholysis in biopsies from consecutively referred PRP cases using multiple-step sections and clinicopathologic correlations.

    Methods: Twenty-three biopsies from 12 consecutively referred patients with classical (type 1) PRP were studied histopathologically. Read More

    Diagnostic utility of Ki-67 and Cyclin D1 immunostaining in differentiation of psoriasis vs. other psoriasiform dermatitis.
    Dermatol Pract Concept 2015 Jul 31;5(3):7-13. Epub 2015 Jul 31.
    Department of Pathology, Acibadem University School of Medicine, Istanbul, Turkey.
    Background: Differentiation of psoriasis from non-psoriasis psoriasiform dermatitis (NPPD) may be difficult for dermatopathologists, as lack of distinctive histopathological features in a subset of cases may cause confusion in diagnosis.

    Objective: As the prototype of psoriasiform dermatitis, psoriasis is a hyperproliferative skin disorder with increased epidermal turnover compared with NPPD, we investigated the role of proliferation markers, Ki-67 and Cyclin D1 as diagnostic tools to differentiate psoriasis from other psoriasiform dermatitis.

    Methods: Histopathological specimens of psoriasis (n = 35) and NPPD (n = 36, 14 pityriasis rubra pilaris, 12 pityriasis rosea and 10 lichen simplex) cases were reviewed and immunohistochemically stained for Ki-67 and Cyclin D1. Read More

    Novel CARD11 Mutations in Human Cutaneous Squamous Cell Carcinoma Lead to Aberrant NF-κB Regulation.
    Am J Pathol 2015 Sep 26;185(9):2354-63. Epub 2015 Jul 26.
    Division of Cancer Research, University of Dundee, Dundee, United Kingdom; Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennsylvania. Electronic address:
    NF-κB signaling plays a crucial role in regulating proliferation and differentiation in the epidermis. Alterations in the NF-κB pathway can lead to skin pathologies with a significant burden to human health such as psoriasis and cutaneous squamous cell carcinoma (cSCC). Caspase recruitment domain (CARD)-containing scaffold proteins are key regulators of NF-κB signaling by providing a link between membrane receptors and NF-κB transcriptional subunits. Read More

    Activating CARD14 Mutations Are Associated with Generalized Pustular Psoriasis but Rarely Account for Familial Recurrence in Psoriasis Vulgaris.
    J Invest Dermatol 2015 Dec 23;135(12):2964-70. Epub 2015 Jul 23.
    St John's Institute of Dermatology, King's College London, London, UK.
    Caspase recruitment family member 14 (CARD14, also known as CARMA2), is a scaffold protein that mediates NF-κB signal transduction in skin keratinocytes. Gain-of-function CARD14 mutations have been documented in familial forms of psoriasis vulgaris (PV) and pityriasis rubra pilaris (PRP). More recent investigations have also implicated CARD14 in the pathogenesis of pustular psoriasis. Read More

    CARD14 Glu138 mutation in familial pityriasis rubra pilaris does not warrant differentiation from familial psoriasis.
    J Dermatol 2016 Feb 1;43(2):187-9. Epub 2015 Jul 1.
    Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan.
    Some familial cases of pityriasis rubra pilaris (PRP) have the CARD14 gene mutations that are also detected in familial psoriasis vulgaris. However, genotype-phenotype correlation in these two entities is poorly understood. Here, we report a case of PRP with a new mutation in CARD14. Read More

    The practical usefulness of dermoscopy in general dermatology.
    G Ital Dermatol Venereol 2015 Oct 18;150(5):533-46. Epub 2015 Jun 18.
    Department of Experimental and Clinical Medicine Institute of Dermatology University of Udine, Udine, Italy -
    Beside to traditional use, dermoscopy is more and more used in the assessment of other "general" dermatologic conditions, namely scalp and hair disorders (trichoscopy), nails abnormalities (onychoscopy), skin infections and infestations (entomodermoscopy), and cutaneous inflammatory diseases (inflammoscopy). Among the list of new applications of dermoscopy, the study of inflammatory dermatoses is probably the most promising topic in terms of development and usefulness, considering the large number of such disorders and the frequent problems in their differential diagnosis which the dermatologist encounters in own daily clinical practice. In this paper, we report selected relatively common clinical differential diagnosis issues concerning inflammatory dermatoses (and some clinically related noninflammatory conditions), analysing them by a dermoscopic point of view in order to assist their noninvasive resolution according to the available literature data and our personal experience, including papulosquamous dermatoses (guttate psoriasis, pityriasis lichenoides chronica, pityriasis rosea, lichen planus, lymphomatoid papulosis, classic pityriasis rubra pilaris, papulosquamous sarcoidosis, disseminated forms of porokeratosis and papulosquamous chronic GVHD), dermatoses presenting with erythematous-desquamative patches/plaques (plaque psoriasis, eczematous dermatitis, pityriasis rosea, mycosis fungoides, subacute cutaneous lupus erythematosus), palmar psoriasis vs. Read More

    Ineffectiveness of tumor necrosis factor-α blockers and ustekinumab in a case of type IV pityriasis rubra pilaris.
    Indian Dermatol Online J 2015 May-Jun;6(3):207-9
    Department of Internal Medicine and Medical Specialities, Pathology Unit, Arcispedale Santa Maria Nuova-IRCCS, Reggio Emilia, Italy.
    Treatment of pityriasis rubra pilaris (PRP) may be difficult since no standardized therapeutic approach has been established. Recently, tumor necrosis factor-α (TNF-α) blockers have been demonstrated to be favorable in the management of recalcitrant PRP. The authors report a case of a patient who presented a type IV PRP or circumscribed, juvenile type. Read More

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