Pityriasis Rubra Research Articles

Pityriasis Rubra Pilaris, Refractory to IL-17A Inhibition, with Rapid Improvement Following IL-17A/IL-17F Blockade by Bimekizumab

Introduction: Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disorder which can severely affect patient quality of life. While there are no FDA-approved therapies for PRP, methotrexate and acitretin are often used as first line treatments. Recent analyses have implicated dysregulation of the IL23/IL17 axis in PRP pathogenesis and the use of biologics for severe cases have been reported in the literature. Case Report: We report the case of a male in his thirties who presented with PRP refractory to a second course of secukinumab. Physical exam revealed diffuse salmon-colored hyperkeratotic patches coalescing to cover his entire body (BSA >95%). The patient was started on combination therapy with acitretin and bimekizumab. The patient recovered rapidly, with near total resolution of his skin findings four weeks after beginning treatment. Discussion: Unlike earlier biologics targeting IL-17, bimekizumab has activity on IL-17F in addition to IL-17AF and IL-17A. The efficacy of bimekizumab in this case may signify an important role for IL-17F in PRP pathogenesis. Bimekizumab may offer improved efficacy for the treatment of pityriasis rubra pilaris (PRP) in patients who do not respond to other therapies.

Erythroderma in the elderly.

Erythroderma is the end-stage condition caused by various inflammatory diseases, presenting with widespread generalized coalesced erythema on the trunk and extremities. Erythroderma is not a disease itself, but rather is a symptom expressing erythrodermic condition, which is frequently associated with inguinal lymphadenopathy, chills, and mild fever. The clinical characteristics include sparing the folds of the trunk and extremities (deck-chair sign), and cobblestone-like disseminated grouping prurigo; however, the deck-chair sign is not specific to papulo-erythroderma (Ofuji disease). Erythroderma is induced by various causes, such as eczema, psoriasis, atopic dermatitis, drug eruption, lymphoma, lichen planus, pityriasis rubra pilaris, autoimmune bullous diseases, graft-versus-host disease, dermatomyositis, internal malignancy, and others. By contrast, it is not uncommon for even thorough investigations to often fail to identify any significant underlying or occult diseases. Such cases are often diagnosed as idiopathic erythroderma. In elderly cases, some regard erythroderma as late-onset atopic dermatitis, even if the patient does not have a history of childhood atopic dermatitis, while others consider it as a distinct condition with immune responses similar to atopic dermatitis. The etiology of erythroderma is suggested to be a Th2-dominant condition with IL-4/IL-13 playing a central role, suggesting that therapies targeting those Th2 molecules may result in sufficient effects. In this review, the characteristics of erythroderma in the elderly and new therapeutic approaches are discussed.

Uncommon Presentation of Pityriasis Rubra Pilaris of the Scalp: Clinical, Trichoscopic, and Histopathologic Features and Review of the Literature

Pityriasis rubra pilaris (PRP) presents a diagnostic challenge due to its varied clinical manifestations and the scarce literature on scalp involvement. This article presents a case report of a 59-year-old female with PRP solely affecting the scalp, detailing its clinical, trichoscopic, and histopathological features. Trichoscopy revealed a novel finding of white-silvery scales forming hair casts with a triangular shape, distinct from the existing literature. A literature review comparing our findings with pertinent articles underscored the uniqueness of our case. We discuss differential diagnoses and treatment options, based on available evidence. Our case highlights the importance of understanding scalp manifestations in PRP, enhancing diagnostic accuracy, and improving treatment strategies for this rare condition. Furthermore, the review of the literature compares our observations with available case reports and case series, outlining differential diagnoses and trichoscopic and histopathological diagnostic approaches to PRP, enriching overall clinical knowledge of PRP.

Pityriasis Rubra Pilaris and underlying malignancy.

A 59-year-old man presented with a five-month history of widespread, salmon-colored, scaly plaques. Skin examination revealed salmon-colored plaques with "islands of sparing" on the trunk and waxy keratoderma on the palms and soles. Histopathology of the lesion showed alternating orthokeratosis and parakeratosis in vertical and horizontal directions, consistent with a diagnosis of pityriasis rubra pilaris (PRP). The patient was unresponsive to methotrexate and Secukinumab. Further evaluation through a computerized tomography scan revealed a presence of air-filled multicystic changes in the upper left lung, accompanied by diffuse pulmonary bullae. Elevated serum tumor markers, including CEA, NSE, and SCC, indicated an underlying acinar-predominant adenocarcinoma, suggesting a paraneoplastic PRP. He was subsequently referred to the oncology department for further management. This report underscores the importance of identifying an underlying malignancy in recalcitrant PRP cases.

Pityriasis rubra pilaris: A review

Pityriasis rubra pilaris (PRP, syn.: Devergie's disease) is a rare idiopathic papulo-squamous inflammatory skin disease characterized by follicular papules, palmar-plantar keratoderma, scaling plaques of orange-red color with characteristic foci of unaffected skin. Histologically, the alternating pattern of orthokeratosis and parakeratosis is considered a distinctive feature of PRP (staggered hyperkeratosis). A violation of the regulation of innate immunity is a component of the pathogenesis of PRP. Congenital mutations of CARD 14 or concomitant antigens, which can take the form of iatrogenic lesions, infections or malignant neoplasms, can be the initial triggers of the disease, although the etiology is often idiopathic. There are classically five subtypes of the disease, depending on the age of onset and clinical picture. Type VI PRP is associated with infection caused by the human immunodeficiency virus. CARD 14-associated papulosquamous rash and discoid dermatitis of the face represent new clinical phenotypes of PRP. Devergie's disease has a pronounced negative impact on the quality of life, and those who become ill are at increased risk of depression and suicide. Ixekizumab, methotrexate and secukinumab can be considered as systemic drugs of the first choice. If there is a contraindication to immunosuppressive therapy, high doses of isotretinoin are recommended. Topical agents, including calcipotriene, calcineurin inhibitors, emollients and topical corticosteroids, are used as additional therapy.

Pityriasis rubra pilaris with eosinophilia in a young patient: a case report

Introduction and importance: Pityriasis rubra pilaris is a rare inflammatory papulosquamous disorder which manifests in six clinical subtypes affecting both pediatric and adult populations. Presentation of case: A 14-year-old female presented with multiple itchy scaly lesions on her hands and legs which began as vesicles 9 days after birth. Histopathological examination confirmed the diagnosis of pityriasis rubra pilaris. Further investigations revealed significant peripheral and tissue eosinophilia. The patient was treated with oral isotretinoin, which resulted in the resolution of the lesions. Case discussion: It is a rare inflammatory papulosquamous disorder characterized by palmoplantar keratoderma and follicular papules coalescing into distinct plaques characterized by a reddish-orange hue and nonadherent flaking scales. The patients with tissue and/or peripheral eosinophilia are usually older at presentation and more likely to have multisite disease. Our patient, in contrast, is a young female which makes this case noteworthy. Conclusion: This unusual finding of eosinophilia in a young patient underscores the necessity for further research and evaluation to enhance understanding of the pathophysiology of pityriasis rubra pilaris.

Wong-Type Dermatomyositis: Literature Review of a Rare Variant.

Wong-type dermatomyositis (WTDM) was first formally discussed in the literature in 1969 by Dr. K.O. Wong. This rare variant of dermatomyositis (DM) is characterized by overlapping features of both classic DM and the cutaneous features of pityriasis rubra pilaris. Since 1969, few cases of WTDM have been published in the literature likely due to the rarity of this condition or lack of recognition by clinicians. This narrative review presents the current published English literature on WTDM, analyzing its clinical presentation, diagnostic testing, and treatments along with a comparison to classic DM. Given the overlap of features of both diseases and patients experiencing a better response to classic DM treatments, our results suggest that WTDM is a rare subtype of DM rather than simply an overlap of pityriasis rubra pilaris and DM presenting in 1 patient. We suggest that clinicians evaluate WTDM patients with very thorough histories, physical examinations, histopathology, and appropriate serological studies and monitor closely for systemic symptoms and development of malignancy. WTDM should be treated using conventional treatments for classical DM. Further studies are needed to understand the pathogenesis of WTDM including more specific and distinguishing autoantibody profiles from classical DM, as well as long-term clinical course of WTDM for best management, including recently available biological treatments.

COVID-19 Vaccination-Induced Pityriasis Rubra Pilaris

Abstract: Amidst the COVID-19 pandemic, the rapid development of vaccines transformed immunization strategies. With 70.6% of the global population vaccinated with at least one dose and 13.53 billion doses administered worldwide, certain dermatological side effects like Pityriasis Rubra Pilaris (PRP) have been linked to the COVID-19 vaccination. This review aims to critically analyze the existing evidence on the correlation between COVID-19 vaccination and PRP, emphasizing its incidence and management strategies. A review of 62 articles was conducted, of which only 19 met the inclusion criteria. Out of these, 16 had complete information necessary for the review. The review identified 16 cases of PRP, predominantly in males (62.5%) with an average age of 59. A significant percentage (44%) of cases occurred approximately 12 days after the third dose (booster). Symptoms commonly included painful rashes, itching, and erythematous, reddish, scaly plaques. Treatments varied, with oral retinoids and monoclonal antibody-based biological therapies being the most common. The importance of continuous monitoring of dermatological side effects, such as PRP, is underscored, especially as the world population will require ongoing booster shots. Standardizing PRP treatment is a critical step toward ensuring optimal patient care.

Off-Label Uses of Abrocitinib: Review of Emerging Therapeutic Applications beyond Atopic Dermatitis.

Abrocitinib, an oral small-molecule Janus Kinase 1 (JAK1) inhibitor, is primarily approved for treating moderate-to-severe atopic dermatitis (AD) in adults and adolescents aged 12 and older. This review examines the emerging off-label uses of Abrocitinib. We identified 37 papers reporting on the use of Abrocitinib in various conditions other than AD. The most commonly reported uses were for vitiligo, prurigo nodularis, and hand eczema, with 12 cases each. There were also 10 cases of lichen sclerosus and chronic pruritus of unknown origin and 5 cases each of pityriasis rubra pilaris alopecia areata. Additionally, erythematotelangiectatic rosacea and steroid-induced rosacea were reported in four cases each. Other conditions treated with Abrocitinib were noted, but these mostly had only one or two reported cases. Interestingly, out of the 103 patients reviewed, all studies reported favorable clinical outcomes and satisfactory results, with the exception of one isolated case where Abrocitinib was used to treat erythematotelangiectatic rosacea.

An insight into the bactericidal and phytochemical properties of leaf extract of Vernonia squarrosa and understanding antibacterial activity by molecular docking

IntroductionNumerous plants in the Vernonia genus are used in Traditional Chinese Medicine (TCM) to treat a wide range of illnesses, such as pityriasis rubra pilaris, infections, wounds, dermatitis, mastitis, diarrhoea, and colds. These genera also have antibacterial, antipyretic, and anti-inflammatory properties. The current work sought to investigate the bactericidal and phytochemical characteristics of Vernonia squarrosa leaf extract and comprehend antibacterial action using molecular docking. MethodsAn in vitro study was carried out to investigate the antibacterial capabilities of V. squarrosa. HRLCMS analysis was conducted to determine the phytoconstituents of the chloroform extract (LC). For molecular docking validation, PyRx, an Open Babel integrated Auto Dock programme, was used. ResultsPresent investigation reveals strong antibacterial activity against two tested gram-positive and gram-negative bacterial strains compared to other solvent extracts, LC at a higher concentration demonstrated a better response. Against Bacillus subtilis (20.05±0.88 mm) and Escherichia coli (20 ± 0.00 mm), the active components of the extracts formed the largest inhibitory zone followed by Pseudomonas aeruginosa (19.50±0.50 mm) and Staphylococcus aureus (14.33 ± 0.33 mm). The LC extract had lower minimum inhibitory concentrations (MIC) and stronger bactericidal activity than the other extracts. DiscussionV. squarrosa's LC showed higher levels of antibacterial activity against the tested bacterial strains than other solvent extracts, most likely as a result of the presence of 31 chemical components, including flavonoids, glycosides, alkaloids, phenolics, and triterpenoids. Developing unique TCM formulations assume significance in global healthcare scenarios to combat bacterial infections and in this context, the current paper underlines the importance of the potent antimicrobial properties of chloroform extract of V. squarrosa. This study revealed that chloroform extract of V. squarrosa has a great deal of antibacterial activity. ConclusionAccording to the study, V. squarrosa leaf chloroform extract possesses antibacterial properties because of its active phytocompounds. Its wide range of bioactive components makes it a prime choice for developing novel formulations to neutralize threats of newly emerging strains of harmful bacteria. The aforementioned discoveries may deepen our comprehension of phenomenal wisdom and deep science associated with the legendary TCM.

Guselkumab - In Psoriasis and Beyond.

Guselkumab is an interleukin 23p19 inhibitor, and the first in this group, to be approved by the US Food and Drug Administration for the management of moderate to severe psoriasis. Apart from its utility in psoriasis, there are a number of other dermatologic conditions where guselkumab has demonstrated value. The aim of this narrative review is to describe the utility of guselkumab in psoriasis as well as its implication in off-label dermatologic disorders. Pubmed, Google Scholar, Scopus and ResearchGate were searched for scholarly articles related to guselkumab and its utility in dermatology using the search terms "Guselkumab" AND "Psoriasis" AND "other dermatological disorders". Guselkumab is a valuable biologic agent for the management of psoriasis and psoriatic arthropathy. It has also been used successfully for other dermatologic disorders like hidradenitis suppurativa, lichen planus, pityriasis rubra pilaris and pyoderma gangrenosum. Recently, its utility in Stewart-Treves angiosarcoma (STA) has been exemplified. Guselkumab usage is not limited to psoriasis. Its benefit extends to many more dermatologic conditions. Its utility in STA could open an avenue for its application in the field of oncology. Furthermore, it has an acceptable safety profile.

Emerging Role of Biologic Drugs Targeting IL-17 and IL-23: Pityriasis Rubra Pilaris.

Pityriasis rubra pilaris (PRP) is a rare, papulosquamous, inflammatory skin disease. PRP represents a therapeutic challenge. The rarity of this disease and its possible spontaneous remission makes the conduction and interpretation of therapeutic studies particularly difficult. Moreover, PRP not infrequently proves resistant to common topical and conventional systemic therapies. In this context, numerous biologic agents have been reported in PRP treatment. The aim of our manuscript was to review the current literature to evaluate the possible role of biologics targeting the IL17/23 axis in PRP management. Recent cases in the literature have highlighted the use of several promising drugs: IL-17 inhibitors, IL-23 inhibitors, and the IL-12/23p40 inhibitor ustekinumab. However, it should be noted that all these drugs are approved for moderate-to-severe plaque psoriasis and their use in PRP is off label. The treatment of PRP is based on clinical experience, case reports or case series reported in the literature, as randomized controlled trials are difficult to conduct due to the rarity of the condition. Despite data on the efficacy of drugs targeting IL-17 and IL-23 being promising, they are still limited. Certainly, further studies are desirable to better characterize PRP and establish shared guidelines.

Loss-of-function mutations in Keratin 32 gene disrupt skin immune homeostasis in pityriasis rubra pilaris

Pityriasis rubra pilaris (PRP) is an inflammatory papulosquamous dermatosis, characterized by hyperkeratotic follicular papules and erythematous desquamative plaques. The precise pathogenic mechanism underlying PRP remains incompletely understood. Herein, we conduct a case-control study involving a cohort of 102 patients with sporadic PRP and 800 healthy controls of Han Chinese population and identify significant associations (P = 1.73 × 10−6) between PRP and heterozygous mutations in the Keratin 32 gene (KRT32). KRT32 is found to be predominantly localized in basal keratinocytes and exhibits an inhibitory effect on skin inflammation by antagonizing the NF-κB pathway. Mechanistically, KRT32 binds to NEMO, promoting excessive K48-linked polyubiquitination and NEMO degradation, which hinders IKK complex formation. Conversely, loss-of-function mutations in KRT32 among PRP patients result in NF-κB hyperactivation. Importantly, Krt32 knockout mice exhibit a PRP-like dermatitis phenotype, suggesting compromised anti-inflammatory function of keratinocytes in response to external pro-inflammatory stimuli. This study proposes a role for KRT32 in regulating inflammatory immune responses, with damaging variants in KRT32 being an important driver in PRP development. These findings offer insights into the regulation of skin immune homeostasis by keratin and open up the possibility of using KRT32 as a therapeutic target for PRP.

Pityriasis Rubra Pilaris Following Administration of the Pfizer-BioNTech COVID-19 Vaccine Successfully Treated with Acitretin and Dupilumab

Introduction: Pityriasis rubra pilaris (PRP) is an inflammatory eruption of unknown origin; rare cases of COVID-19 vaccine-induced PRP have been reported. Here, we present a case of COVID-19 vaccine-induced pityriasis rubra pilaris inadequately managed with acitretin, successfully treated with the IL-4/IL-13 inhibitor dupilumab. Case Report: A 76-year-old male presented to an outpatient dermatology clinic with a 2-month history of a profoundly pruritic worsening rash characterized by large, orange-salmon-colored follicular papules with scale coalescing into plaques covering approximately 80% of the body surface area. The plaques had well-defined borders and multiple islands of sparing characteristic of PRP. Multiple therapies were trialed with no improvement, including oral prednisone, mycophenolate, topical corticosteroids, antiparasitics, antifungals, doxepin, and UVB treatments. A trial of oral acitretin resulted in improvement of the skin plaques and keratoderma, but the patient remained uncontrollably pruritic. Dupilumab was initiated which provided rapid relief, and the patient has remained clear for several months. Conclusion: Clinicians should be aware of dupilumab’s potential for effective treatment of PRP with recalcitrant pruritus.

Psoriasis in pediatric dermatological clinical practice: diagnosis and management

The relevance of this topic is confirmed by the widespread psoriasis prevalence. Psoriasis in children has severe forms growth and diagnostic difficulties. It is crucially important to examine histologically the skin especially in pediatric dermatological clinical practice. According to our observations Pityriasis Rubra Pilaris has become one of the most frequent dermatoses with which differential diagnosis of psoriasis has to be carried out. A clinical dermatosis observation in a 13-year-old girl is described. The disease was similar to the manifestations of Pityriasis Rubra Pilaris. The results of the affected skin biopsy study did not exclude this diagnosis. The clinical and morphological manifestations of the dermatosis could be effected by steroid therapy. Subsequent dynamic observation and histological examination allowed us to diagnose psoriasis. Current data is collected and presented. The choice of external therapy in pediatric practice is a controversial topic. Clinical results of the Activated Zinc Pyrithion external forms use in the psoriasis treatment are presented.

Targeting IL-1 controls refractory pityriasis rubra pilaris.

Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disease with a poorly understood pathogenesis. Through a molecularly driven precision medicine approach and an extensive mechanistic pathway analysis in PRP skin samples, compared to psoriasis, atopic dermatitis, healed PRP, and healthy controls, we identified IL-1β as a key mediator, orchestrating an NF-κB-mediated IL-1β-CCL20 axis, including activation of CARD14 and NOD2. Treatment of three patients with the IL-1 antagonists anakinra and canakinumab resulted in rapid clinical improvement and reversal of the PRP-associated molecular signature with a 50% improvement in skin lesions after 2 to 3 weeks. This transcriptional signature was consistent with in vitro stimulation of keratinocytes with IL-1β. With the central role of IL-1β underscoring its potential as a therapeutic target, our findings propose a redefinition of PRP as an autoinflammatory keratinization disorder. Further clinical trials are needed to validate the efficacy of IL-1β antagonists in PRP.

JAAD Game Changers: CARD14-associated papulosquamous eruption: A spectrum including features of psoriasis and pityriasis rubra pilaris

JAAD Game Changers: CARD14-associated papulosquamous eruption: A spectrum including features of psoriasis and pityriasis rubra pilaris

Differential diagnosis of erythemato-squamous diseases using a hybrid ensemble machine learning technique

Erythemato-squamous Diseases (ESD) encompass a group of common skin conditions, including psoriasis, seborrheic dermatitis, lichen planus, pityriasis rosea, chronic dermatitis, and pityriasis rubra pilaris. These dermatological conditions affect a significant portion of the population and present a current challenge for accurate diagnosis and classification. Traditional classification methods struggle due to shared characteristics among these diseases. Machine Learning offers a valuable tool for aiding clinical decision-making in ESD classification. In this study, we leverage the UC Irvine (UCI) dermatology dataset by applying necessary preprocessing steps to handle missing data. We conduct a comparative analysis of two feature selection methods: One-way ANOVA and Chi-square test. To enhance the model’s performance, we employ hyper-parameter tuning through GridSearchCV. The training process encompasses various algorithms, including Support Vector Machine (SVM), Logistic Regression, k-Nearest Neighbors (kNN), and Decision Trees. The culmination of our work is a hybrid ensemble machine learning model that combines the strengths of the trained classifiers. This ensemble classifier achieves an impressive accuracy of 98.9% when validated using a 10-fold cross-validation approach.

Role of IL-23 inhibitors including risankizumab and guselkumab in the treatment of pityriasis rubra pilaris.

Pityriasis rubra pilaris (PRP) is a rare and chronic inflammatory dermatologic condition characterized by hyperkeratotic salmon-colored plaques and palmoplantar keratoderma. Traditional therapeutic modalities have shown limited efficacy and often entail potential adverse effects, highlighting the need for alternative treatment options. Our review aims to summarize the current evidence on the off-label use of IL-23 inhibitors, risankizumab and guselkumab, in the treatment of PRP. These biologic agents have been approved for psoriasis, and their potential role in managing PRP has recently garnered interest. We conducted a comprehensive literature search on PubMed and Scopus databases, identifying relevant studies published in English up to June 2023 following PRISMA guidelines. A total of 10 studies were selected for data extraction and review. Results from the selected studies demonstrated encouraging outcomes with both risankizumab and guselkumab in managing PRP. Among 11 patients treated with risankizumab, 10 showed notable improvements in various disease manifestations, including pruritus, erythema, and affected body surface area. DLQI scores and BSA percentages reported a significant improvement before and after risankizumab treatment (p = 0.0322; p = 0.0216). However, two cases also reported symptom aggravation or even disease worsening. Patients treated with guselkumab exhibited ultimate improvement in all five cases, with complete clearance in three out of five cases. DLQI and BSA percentages also reported significant improvement with treatment with guselkumab (p = 0.0172; p < 0.0001). While most cases demonstrated positive outcomes, there were isolated instances of worsening symptoms, emphasizing the need for caution and further investigation. Further research with larger sample sizes and longer follow-up periods is necessary to establish the efficacy, optimal dosing, and long-term safety of risankizumab and guselkumab in treating PRP. Overall, we provide valuable insights into the potential use of IL-23 inhibitors, risankizumab, and guselkumab, as promising treatment options for PRP. These biologics have shown efficacy in improving symptoms in treatment-resistant cases, offering new avenues for clinicians to explore in the treatment of PRP.

Surgical Management of Severe Cicatricial Ectropion Secondary to Pityriasis Rubra Pilaris.

Surgical Management of Severe Cicatricial Ectropion Secondary to Pityriasis Rubra Pilaris.