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    746 results match your criteria Pityriasis Rubra Pilaris

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    Autoinflammatory keratinization diseases: An emerging concept encompassing various inflammatory keratinization disorders of the skin.
    J Dermatol Sci 2018 Feb 1. Epub 2018 Feb 1.
    Department of Dermatology, Fujita Health University School of Medicine, Toyoake, Japan.
    Classifying inflammatory skin diseases is challenging, especially for the expanding group of disorders triggered by genetic factors resulting in hyperactivated innate immunity that result in overlapping patterns of dermal and epidermal inflammation with hyperkeratosis. For such conditions, the umbrella term "autoinflammatory keratinization diseases" (AIKD) has been proposed. AIKD encompasses diseases with mixed pathomechanisms of autoinflammation and autoimmunity, and includes IL-36 receptor antagonist (IL-36Ra)-related pustulosis, CARD14-mediated pustular psoriasis, pityriasis rubra pilaris (PRP) type V, and familial keratosis lichenoides chronica (KLC). Read More

    Methotrexate Treatment for Pityriasis Rubra Pilaris: A Case Series and Literature Review.
    Acta Derm Venereol 2018 Jan 16. Epub 2018 Jan 16.
    Department of Dermatology, Medical University of Graz, AT-8036 Graz, Austria.
    Treatment recommendations for pityriasis rubra pilaris (PRP) are based solely on case reports and small case series, as to-date no randomized controlled trials are available. We present here a case series of 3 patients and a literature review of 28 studies treating a total of 116 patients, with the aim of providing data regarding efficacy and safety of methotrexate in the treatment of PRP. Methotrexate was effective in our patients; the review showed an overall response rate of 65. Read More

    A Review on Pityriasis Rubra Pilaris.
    Am J Clin Dermatol 2018 Jan 4. Epub 2018 Jan 4.
    National Skin Centre, 1 Mandalay Rd, Singapore, 308205, Republic of Singapore.
    Pityriasis rubra pilaris (PRP) is an idiopathic, papulosquamous inflammatory dermatosis. It is characterized by hyperkeratotic follicular papules coalescing into orange-red scaly plaques, islands of sparing, and palmoplantar keratoderma. PRP can be subdivided into six clinical subtypes according to Griffiths' classification, based on age of onset, disease extent, prognosis, and other associated features. Read More

    Lakartidningen 2017 Nov 21;114. Epub 2017 Nov 21.
    Karolinska Universitetssjukhuset Hudkliniken - Stockholm, Sweden Karolinska Universitetssjukhuset Hudkliniken - Stockholm, Sweden.
    Psoriasis in childhood and adolescence Psoriasis is a common inflammatory disease affecting 2-3% of the worldwide population. Onset in childhood and adolescence is not uncommon and around 30% of patients report onset before 20 years of age. Childhood psoriasis has been shown to differ in clinical presentation and in underlying genetics compared to adult psoriasis patients. Read More

    [Pityriasis rubra pilaris].
    Ann Dermatol Venereol 2018 Jan 28;145(1):50-59. Epub 2017 Dec 28.
    Service de dermatologie, hôpitaux universitaires de Genève (HUG), 1211 Genève, Suisse.
    Pityriasis rubra pilaris is a rare heterogeneous dermatosis associating three clinical signs to different degrees: follicular corneal papules, reddish-orange palmoplantar keratoderma and erythematosquamous lesions that may in some cases be very extensive, interspersed with patches of healthy skin. The aetiology is unclear, and in most cases, the trigger factors consist of trauma or infection, probably in subjects with an existing predisposition. In other cases, the condition is associated with immunological disorders or, in familial cases, genetic keratinisation abnormalities similar to ichthyosis. Read More

    Pityriasis rubra pilaris: algorithms for diagnosis and treatment.
    J Eur Acad Dermatol Venereol 2017 Dec 15. Epub 2017 Dec 15.
    Department of Dermatology and Allergy Biederstein, Technische Universität München, Germany, Munich.
    Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disease that affects men and women of all ages and also children. The clinical appearance of PRP is highly variable, as is the individual prognosis. Therefore, stratifying PRP into six disease subtypes represents a first step to personalized medicine for this rare inflammatory skin disease. Read More

    Management of refractory pityriasis rubra pilaris: challenges and solutions.
    Clin Cosmet Investig Dermatol 2017 9;10:451-457. Epub 2017 Nov 9.
    Institute of Dermatology, Fondazione Policlinico Universitario A. Gemelli, Catholic University of the Sacred Heart, Rome, Italy.
    Pityriasis rubra pilaris (PRP) is a rare chronic inflammatory papulosquamous skin disease. Its clinical presentation and evolution is very variable. The most frequent clinical features are follicular papules, progressing to yellow-orange erythroderma with round small areas of normal skin and the well-demarcated palmoplantar keratoderma. Read More

    Ichthyosiform Pityriasis Rubra Pilaris-Like Eruption Secondary to Ponatinib Therapy: Case Report and Literature Review.
    Drug Saf Case Rep 2017 Nov 14;4(1):19. Epub 2017 Nov 14.
    Department of Dermatology and Cutaneous Surgery, Jackson Memorial Hospital, University of Miami Miller School of Medicine, 1475 NW 12th Avenue, Miami, FL, 33136, USA.
    Tyrosine kinase inhibitors have revolutionized the chemotherapy arena as targeted therapies for a multitude of malignancies. They are more selective than conventional chemotherapy, and often elicit fewer systemic adverse events, however toxicities still exist. Cutaneous toxicities are common and their management presents a novel challenge to physicians and patients. Read More

    Ustekinumab treatment of pityriasis rubra pilaris: A report of five cases.
    J Dermatol 2018 Feb 28;45(2):202-206. Epub 2017 Oct 28.
    I Dermatology Division, Istituto Dermopatico dell'Immacolata (IDI)-IRCCS-FLMM, Rome, Italy.
    Pityriasis rubra pilaris (PRP) is a rare, chronic, inflammatory skin disease of unknown etiology. Patients refractory to conventional therapies have been treated successfully with biologic drugs such as anti-tumor necrosis factor agents. Recently, a role of the interleukin-23/T-helper 17 axis in PRP has been described. Read More

    Methotrexate treatment in a case of juvenile pityriasis rubra pilaris.
    Pediatr Dermatol 2018 Jan 17;35(1):e62-e63. Epub 2017 Oct 17.
    Department of Pediatric Dermatology, University of Minnesota, Minneapolis, MN, USA.
    An 8-year-old boy who was initially diagnosed with plaque psoriasis failed management with topical therapies and skin biopsy confirmed the suspected diagnosis of juvenile pityriasis rubra pilaris (PRP). Pityriasis rubra pilaris is a rare inflammatory disorder of the skin characterized by follicular keratotic papules coalescing into plaques, along with palmoplantar keratoderma. Treatment modalities include topical and systemic therapies, although previous studies have not shown much benefit with methotrexate in children. Read More

    Refractory pityriasis rubra pilaris treated with etanercept, adalimumab, or ustekinumab: A retrospective investigation.
    Dermatol Ther 2017 Nov 15;30(6). Epub 2017 Oct 15.
    Division of Dermatology, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.
    Pityriasis rubra pilaris (PRP) is a rare, difficult to treat papulosquamous disorder that responds variably to retinoids and immunosuppression. Successful use of biologics for treating PRP has been described in the literature by case reports and a limited number of case series. To provide additional data, we retrospectively analyzed cases of PRP treated with biologics at our institution. Read More

    Acute generalized exanthematous pustulosis and polyarthritis associated with a novel CARD14 mutation.
    Australas J Dermatol 2018 Feb 3;59(1):e70-e73. Epub 2017 Aug 3.
    Department of Dermatology, Hospital Clinic, Instituto de Investigaciones Biomédicas August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
    Acute generalised exanthematous pustulosis (AGEP) is a rare toxicoderma characterised by an acute onset rash, with many sterile pustules on the surface, high fever and increased acute phase reactants. We report the case of a patient who presented to the dermatology department with an AGEP and polyarthritis, in which a novel CARD14 mutation was identified. The pathophysiological mechanism of AGEP remains unclear, although mutations in the IL36RN gene have been identified in a small subset of AGEP patients. Read More

    Autoinflammatory diseases in dermatology: DITRA and CAMPS.
    Nihon Rinsho Meneki Gakkai Kaishi 2017 ;40(3):169-173
    Department of Dermatology, Fujita Health University School of Medicine.
    Deficiency of interleukin thirty-six receptor antagonist (DITRA) and CARD14 mediated psoriasis (CAMPS) are autoinflammatory diseases in dermatology. The causative genes of DITRA and CMAPS have been identified recently. In this paper, IL36RN and CARD14, the causative gene for DITRA and CAMPS, respectively were explained. Read More

    Eruptive Seborrheic Keratoses Restricted to Plaque/Patch-Stage Mycosis Fungoides.
    Case Rep Dermatol 2017 May-Aug;9(2):35-39. Epub 2017 Jun 8.
    Department of Dermatology, CHU Sart Tilman, University of Liège, Liège, Belgium.
    Eruptive seborrheic keratoses (ESK) are rare in dermatology. They are usually inflammatory in nature and may be encountered as Leser-Trélat sign. ESK may also be simultaneously observed with hepatic angiomas, chemotherapy, segmental neurofibromatosis, HIV or erythrodermic pityriasis rubra pilaris, psoriasis, and drug eruption. Read More

    Efficacy of Triaging Direct Immunofluorescence in Intraepidermal Bullous Dermatoses.
    Am J Dermatopathol 2018 Jan;40(1):24-29
    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, U.K.
    Pityriasis rubra pilaris (PRP) represents a group of rare chronic inflammatory skin disorders in which around one 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), which activates the noncanonical nuclear factor (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 patients. 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

    [Pityriasis rubra pilaris].
    Rev Med Suisse 2016 Mar;12(512):653-7
    Pityriasis rubra pilaris is a rare heterogeneous disorder characterized by follicular keratosis, perifollicular erythema and palmoplantar hyperkeratosis. The aetiology is still unknown. In the majority of cases some triggering factors are found such as trauma or bacterial infection, possibly on a predisposed condition. 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

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