Pityriasis Rubra Research Articles

Paraneoplastische Hauterkrankungen

Various specific skin alterations can occur in patients with malignant diseases. If these skin diseases occur as associated symptoms of amalignant process, they are called paraneoplastic. In this overview, obligate and frequent facultative paraneoplastic skin diseases are assigned according to the triggering type of malignancy. Some of the processes predominantly show alink with malignant diseases of the digestive tract, e.g. acanthosis nigricans, florid cutaneous papillomatosis, necrolytic migratory erythema, Leser-Trélat syndrome, palmoplantar keratoderma, panniculitis and pityriasis rubra pilaris. Others are predominantly associated with ahematolymphoid malignoma, e.g. acquired ichthyosis, exfoliative erythroderma, necrobiotic xanthogranuloma, paraneoplastic pemphigus, plane xanthoma, pyoderma gangrenosum, scleromyxedema, Sweet syndrome and leukocytoclastic vasculitis. In athird group paraneoplastic skin diseases are pooled in association with other malignancies, e.g. Trousseau's syndrome, dermatomyositis, erythema gyratum repens, hypertrichosis lanuginosa acquisita and papuloerythroderma of Ofuji. In order to initiate targeted diagnostics for detection of an underlying malignant disease, it is essential that accomplished physicians recognize the skin diseases that represent obligate or potential paraneoplasms as such.

Guselkumab in the Treatment of Pityriasis Rubra Pilaris (PRP)

Guselkumab in the Treatment of Pityriasis Rubra Pilaris (PRP)

Classification of Skin Disease using Ensemble Data Mining Techniques.

Objective:Skin diseases are a major global health problem associated with high number of people. With the rapid development of technologies and the application of various data mining techniques in recent years, the progress of dermatological predictive classification has become more and more predictive and accurate. Therefore, development of machine learning techniques, which can effectively differentiate skin disease classification, is of vast importance. The machine learning techniques applied to skin disease prediction so far, no techniques outperforms over all the others. Methods:In this research paper, we present a new method, which applies five different data mining techniques and then developed an ensemble approach that consists all the five different data mining techniques as a single unit. We use informative Dermatology data to analysis different data mining techniques to classify the skin disease and then, an ensemble machine learning method is applied. Results:The proposed ensemble method, which is based on machine learning was tested on Dermatology datasets and classify the type of skin disease in six different classes like include C1: psoriasis, C2: seborrheic dermatitis, C3: lichen planus, C4: pityriasis rosea, C5: chronic dermatitis, C6: pityriasis rubra. The results show that the dermatological prediction accuracy of the test data set is increased compared to a single classifier. Conclusion:The ensemble method used on Dermatology datasets give better performance as compared to different classifier algorithms. Ensemble method gives more accurate and effective skin disease prediction.

Efficacy of methotrexate as anti-inflammatory and anti-proliferative drug in dermatology: Three case reports.

Methotrexate (MTX) is a folic acid analog with anti-proliferative (anti-neoplastic, cytotoxic), immunosuppressive and anti-inflammatory properties, which has been used in the treatment of various cutaneous disorders, such as psoriasis, keratoacanthoma, pityriasis rubra pilaris, atopic dermatitis, mycosis fungoides, bullous skin diseases, systemic sclerosis, morphea, lupus erythematosus, dermatomyositis and crusted scabies. Inhibition of cell proliferation is explained through its role in blocking DNA/RNA synthesis, by inhibiting dihydrofolate reductase, necessary for the production of pyrimidine and purine nucleotides. An anticancer effect can be related to α-oxoaldehyde metabolism (MTX increases methylglyoxal levels). Its anti-inflammatory property is based on the inhibition of 5-aminoimidazole-4-carboxamide ribonucleotide transformylase, thus increasing intracellular and extracellular adenosine, a purine nucleoside with anti-inflammatory effect. This drug can limit inflammation by scavenging free radicals and decreasing malondialdehyde-acetaldehyde protein-adduct production. Moreover, the anti-proliferative and anti-inflammatory effects can also be related to inhibition of the DNA methylation pathway, thus inhibiting methionine formation. The aim of the present study was to report various dermatological cases from our daily practice that demonstrate the efficacy of MTX in the treatment of cutaneous diseases, highlighting different mechanisms of action: its anti-inflammatory effect in psoriasis and its anti-proliferative, and anti-neoplastic effect in well-differentiated squamous cell carcinoma or in keratoacanthoma. Moreover, different administration pathways and doses are addressed. Assessment of the treatment plan, clinical improvement of cutaneous lesions, biologic evaluation, final aesthetic result, quality of life, as well as potential adverse effects and drug tolerance related to each case mentioned.

611 Pityriasis rubra pilaris (PRP) treated with ixekizumab: Interim analysis of an open-label trial

611 Pityriasis rubra pilaris (PRP) treated with ixekizumab: Interim analysis of an open-label trial

The management and genetic background of pityriasis rubra pilaris: a single‐centre experience

Pityriasis rubra pilaris (PRP) is a rare chronic inflammatory dermatosis with multifactorial aetiology. It is known that particular caspase recruitment domain family member 14 (CARD14) gene mutations are associated with familial PRP and certain forms of psoriasis. Additionally, few data are available about the role of CARD14 gene variants in sporadic PRP. The clinical picture is variable for the different types of PRP, therefore choosing the adequate treatment is often difficult, furthermore there are no specific guidelines for therapy. Our aim was to survey the efficacy of the applied therapies and to screen the CARD14 gene variants in our PRP patients. In this retrospective study, patients diagnosed with PRP between 2006 and 2016 at our clinic were involved. Besides the follow-up study of the treatments, the genetic analysis of CARD14 gene was performed. We analysed 19 patients, among whom 17 were diagnosed with type I, one with type III, and one with type V PRP. The majority of the patients were successfully treated with acitretin in combination with systemic corticosteroids, and the remaining patients were treated with other systemic therapies with diverse effects. The genetic screening of CARD14 gene revealed two previously described mutations (rs114688446, rs117918077) and six polymorphisms (rs28674001, rs2066964, rs34367357, rs11653893, rs11652075, rs2289541). Ten of 19 patients carried different CARD14 genetic variants either alone or in combination. Based on our experience, we propose that acitretin and an initial combination of short-term systemic corticosteroid therapy could be a successful treatment option for PRP. Although we identified several CARD14 variants in almost half of our cases, we did not find a correlation between the therapeutic response and the genetic background. Our data support the previous observation that CARD14 genetic variants are not specific to PRP, although they may indicate chronic inflammation.

Pityriasis rubra pilaris: A study evaluating patient quality of life in 2 populations

Pityriasis rubra pilaris: A study evaluating patient quality of life in 2 populations

Skin Diseases Prediction: Binary Classification Machine Learning and Multi Model Ensemble Techniques

Unlike many other diseases, the skin disease has more irritability. Dermatology sicknesses incorporates normal skin rashes to serious skin contaminations, which happens because of scope of things, like diseases, warm, allergens, framework issue and drugs. First regular skin issue are dermatitis. Atopic dermatitis is relating current (perpetual) condition that causes eager, aroused skin. Most much of the time it appears as patches on the face, neck, trunk or appendages. It will in general erupt sporadically so die down for a period. A large portion of the dermatological sicknesses are not reparable but rather most the treatments depend on the administration of the side effects related with it. The focus of this research will be the Dermatology database. The problem is to determine the type of Eryhemato-Squamous disease like psoriasis, seboreic dermatitis, lichen planus, pityriasis rosea, cronic dermatitis and pityriasis rubra pilaris. The differential analysis of erythemato-squamous maladies is a genuine issue in dermatology. They all offer the clinical highlights of erythema and scaling, with next to no distinctions. Each pattern is a set of 33 numbers in the range linear values and one of them is nominal. The 80% of the dataset utilize for demonstrating and keep down 20% for approval. Objective is to accomplish best performer algorithm which will convey in dermatology informational collection so for this reason the gut feel recommends distance based calculations like k-Nearest Neighbors and Support Vector Machines may progress admirably. By using 10-fold cross validation and assess calculations utilizing the accuracy metric.

Erythroderma in the course of pityriasis rubra pilaris in a 7-year-old female.

Erythroderma in the course of pityriasis rubra pilaris in a 7-year-old female.

Systemic therapies of pityriasis rubra pilaris: a systematic review.

Pityriasis rubra pilaris (PRP) is a rare papulosquamous disorder. Treatment is challenging; the armamentarium consists of topical corticosteroids, phototherapy, classic systemic treatments such as retinoids or immunosuppressive drugs, and most recently biologicals. However, the relative effectiveness of therapies is unclear. Our objective was to review the published literature on systemic treatment of PRP. A systematic review was conducted on PubMed and the Cochrane Library up to 5 September 2017. Studies evaluating any systemic treatments of PRP (except for historical treatments) were included. Overall, 182 studies met the predefined inclusion criteria, and reported on 475 patients and 652 courses of treatment. 42.0% (225/514) of all patients treated with retinoids achieved an excellent response (isotretinoin: 61.1% [102/167], etretinate: 47% [54/115], and acitretin: 24.7% [43/174]) compared to an excellent response rate of 33.1% (53/160) with methotrexate. Therapy with biologicals was successful in 51.0% of patients (71/133) (ustekinumab: 62.5% [10/16], infliximab: 57.1% [28/49], etanercept: 53.3% [16/30], and adalimumab: 46.4% [13/28]). This review balances effectiveness, side effects, experience, and drug costs in order to suggest a treatment regimen starting with isotretinoin as first-line, methotrexate as second-line and biologicals as third-line treatment for this difficult-to-treat dermatosis.

Distinguishing histopathologic features of acantholytic dermatoses and the pattern of acantholytic hypergranulosis.

Acantholysis can be seen in multiple skin diseases. Adnexal acantholysis has been regarded as a feature distinguishing pemphigus vulgaris (PV) from acantholytic conditions. A retrospective review of the histopathologic features of diseases with acantholysis including PV, pemphigus foliaceus (PF), Hailey-Hailey disease (HHD), Darier disease (DD), Grover disease, and pityriasis rubra pilaris (PRP) was performed. Biopsies of PV (n = 49), HHD (n = 27), DD (n = 25), Grover disease (n = 65), and PRP (n = 33) showed suprabasilar acantholysis. Acantholysis was limited to the lower epidermis in PV and PRP, and involved all epidermal layers in HHD, DD, and Grover disease. Acantholysis in PF (n = 38) mainly involved the upper epidermis. Follicular acantholysis occurred more frequently in PV and PF (P < 0.0001). Eccrine acantholysis was found in PV (42%), HHD (18%), PF (13%), and DD (4%). Grover disease, DD, and HHD had greater dyskeratosis (P < 0.0001). Neutrophils were more common in PV, PF, and HHD, while eosinophils were more common in Grover disease and DD. A pattern termed acantholytic hypergranulosis occurred predominantly in PF. Adnexal acantholysis does not reliably distinguish PV from PF. The level of acantholysis, degree of dyskeratosis, and acantholytic hypergranulosis are distinguishing features between the two types of pemphigus and other acantholytic disorders.

Differentiation of pityriasis rubra pilaris from plaque psoriasis by dermoscopy.

BackgroundDiagnosing and differentiating pityriasis rubra pilaris (PRP) from other disorders can be a challenging task. Although histopathology remains the gold standard, it may not be feasible at times, especially in children. Being noninvasive, dermoscopy can be of great diagnostic importance in such a scenario. Dermoscopy overcomes the refractive properties of stratum corneum by interface medium or cross-polarization enabling easier visualization of lesions.ObjectiveTo study the dermoscopic features of PRP and compare them with dermoscopic features of psoriasis.Patients and MethodsRetrospective observational analysis of dermoscopic characteristics of 28 patients with PRP (16) and psoriasis (12). Polarized dermoscopy at 10× magnification was performed and photographs were captured by Apple iPhone 7. The selection of dermoscopic variables was based on preexisting literature on the dermoscopic patterns of the 2 entities.ResultsThe most frequent dermoscopic criteria of PRP were the presence of a central hair (16/16; 100%), follicular plugs (11/16; 68.7%), and perifollicular yellow/orange halos (9/16; 56.2%). No vascular structures were seen in our cases of PRP. Psoriasis was characterized by regularly distributed dotted vessels (12/12; 100%) and white scales (8/12; 66.6%).ConclusionsRound-to-oval yellowish areas surrounding a central hair with or without follicular plugs represents the most frequent dermoscopic pattern of PRP.

A Practical Approach to the Diagnosis and Treatment of Adult Erythroderma

Erythroderma is an inflammatory skin syndrome that involves desquamation and erythema of more than 90% of the body surface area. It represents a final clinical endpoint for many adult dermatological conditions. The most frequent cause of erythroderma is psoriasis followed by eczematous conditions, drug-induced reactions, pityriasis rubra pilaris and cutaneous T-cell lymphomas. Diagnostic approach must include a thorough history and clinical examination. If the etiology of erythroderma is uncertain multiple skin biopsies may enhance diagnostic accuracy. The initial management of erythroderma must include a nutrition expert evaluation, fluid imbalance assessment, maintaining skin barrier function, sedative antihistamines and exclusion of secondary bacterial infection. We present a practical review of the etiology, diagnosis, and treatment of this entity.

Eritrodermia en el adulto: un enfoque práctico para el diagnóstico y tratamiento

Erythroderma is an inflammatory skin syndrome that involves desquamation and erythema of more than 90% of the body surface area. It represents a final clinical endpoint for many adult dermatological conditions. The most frequent cause of erythroderma is psoriasis followed by eczematous conditions, drug-induced reactions, pityriasis rubra pilaris and cutaneous T-cell lymphomas. Diagnostic approach must include a thorough history and clinical examination. If the etiology of erythroderma is uncertain multiple skin biopsies may enhance diagnostic accuracy. The initial management of erythroderma must include a nutrition expert evaluation, fluid imbalance assessment, maintaining skin barrier function, sedative antihistamines and exclusion of secondary bacterial infection. We present a practical review of the etiology, diagnosis, and treatment of this entity.

Treatment of Pityriasis Rubra Pilaris with Adalimumab: A Case Report

Pityriasis Rubra Pilaris (PRP) is a rare inflammatory dermatosis of unknown etiology and variable presentation. Common clinical features include hyperkeratotic follicular papules, palmoplantar hyperkeratosis and well-demarcated red-orange scaly plaques interspersed with islands of unaffected skin. A limited body of anecdotal evidence predominantly supports the use of systemic retinoids and/or methotrexate in treating the disease. In a subset of these patients, there has been a documented role for biologic tumor necrosis factor (TNF) inhibitors, especially in patients who have failed retinoid or antimetabolite therapy. We present a case of a woman whose PRP responded favorably to adalimumab, highlighting the role of TNF inhibitors and the need for their continued exploration in the treatment of PRP.

Cirmcumscribed juvenile pityriasis rubra pilaris in a 5-year-old-girl treated with topical keratolytic and steroid

Cirmcumscribed juvenile pityriasis rubra pilaris in a 5-year-old-girl treated with topical keratolytic and steroid

Nuclear Factor κB Activation in a Type V Pityriasis Rubra Pilaris Patient Harboring Multiple CARD14 Variants.

Pityriasis rubra pilaris (PRP) is a rare papulosquamous skin disorder, which is phenotypically related to psoriasis. Some familial PRP cases show autosomal dominant inheritance due to CARD14 mutations leading to increased nuclear factor κB (NFκB) activation. Moreover, CARD14 polymorphisms have also been implicated in sporadic PRP. A Hungarian PRP patient with childhood onset disease showing worsening of the symptoms in adulthood with poor therapeutic response underwent genetic screening for the CARD14 gene, revealing four genetic variants (rs117918077, rs2066964, rs28674001, and rs11652075). To confirm that the identified genetic variants would result in altered NFκB activity in the patient, functional studies were carried out. Immunofluorescent staining of the NFκB p65 subunit and NFκB-luciferase reporter assay demonstrated significantly increased NFκB activity in skin samples and keratinocytes from the PRP patient compared to healthy samples. Characterization of the cytokine profile of the keratinocytes and peripheral blood mononuclear cells demonstrated that the higher NFκB activation in PRP cells induces enhanced responses to inflammatory stimuli. These higher inflammatory reactions could not be explained solely by the observed CARD14 or other inflammation-related gene variants (determined by whole exome sequencing). Thus our study indicates the importance of investigations on other genetic factors related to PRP and their further functional characterization to bring us closer to the understanding of cellular and molecular background of disease pathogenesis.

Un nouveau cas d’éruption de type pityriasis rubra pilaire associée à l’inhibiteur de tyrosine kinase ponatinib

Pityriasis rubra pilaris (PRP) is a cutaneous syndrome of unknown origin. Most cases are sporadic and acquired. Herein we report a fifth case of PRP-like eruption associated with ponatinib, a tyrosine kinase inhibitor (TKI). A 60-year-old woman presented at the dermatology department with an erythemato-squamous eruption present for 2weeks. The patient was also being treated in haematology for recurrence of acute lymphoblastic leukaemia. Treatment with ponatinib had been initiated 6weeks earlier. Despite the low specific cutaneous histology, a diagnosis of induced PRP-like eruption was made based on the characteristic clinical aspect. Treatment with local corticosteroids resolved the eruption. The literature contains 6 reported cases of PRP-like eruptions associated with TKI, including 4 with ponatinib. The eruption began from 2weeks to 2-3 months after treatment induction. Prescribed topical corticosteroids have yielded mixed results. A better understanding of the physiopathology of these eruptions associated with TKI could shed light on the pathogenic mechanisms in relation to idiopathic PRP.

Pathologies rares pouvant simuler une spondyloarthrite ou chevaucher avec celle-ci

When a spondyloarthitis is suspected, clinicians have to first identify signs and symptoms from other diseases with similar clinical and imaging features. Identify differential diagnosis has a crucial importance to provide the most appropriate treatment to the patients. In this review, we will examine the clinical signs and imaging features of the disorders, which can mimic or overlap with a spondyloarthritis. We will describe their characteristic symptoms and situations in which we have to think about them. In this review, we will talk about: sacroiliac joint osteoarthritis and osteitis condensans ilii; diffuse and sacroiliac pain in joint hypermobility (Elhers-Danlos); hypo- and hyperparathyroidism; entheseal pain in primary Sjögren's syndrome; sacroiliitis of connective tissue diseases (lupus, etc.) and skeletal manifestations of Behçet's disease, relapsing polychondritis, and sarcoidosis; axial joint involvement of Whipple's disease; rheumatism associated with pityriasis rubra-pilaris; PAPA and PASS syndromes; fibrodysplasia ossificans progressiva; paraneoplastic spondyloarthritis; hypophosphatemia; calcineurin inhibitor-induced pain syndromes (CIPS); skeletal manifestations of fluorosis; pachydermoperiostosis and joint involvement in adult Kawasaki disease.

Erythrodermic pityriasis rubra pilaris managed at home: intensive community care followed by ustekinumab

Erythrodermic pityriasis rubra pilaris managed at home: intensive community care followed by ustekinumab