Treatment Of Pityriasis Rubra Pilaris Research Articles

Guselkumab for Pityriasis Rubra Pilaris and Dysregulation of IL-23/IL-17 and NFkB Signaling: A Nonrandomized Trial.

There is no US Food and Drug Administration-approved treatment for pityriasis rubra pilaris (PRP), and it is common for patients to fail to experience improvement with several systemic options. Involvement of interleukin (IL) 23 suggests a potential therapeutic target. To determine whether guselkumab, an IL-23p19 inhibitor, provides clinical improvement for participants with PRP and better understand gene and protein dysregulation in PRP. This single-arm, investigator-initiated nonrandomized trial was conducted from October 2019 to August 2022 at a single-center academic university with participants from 8 states in the US. In total, 14 adults with moderate to severe PRP were enrolled; 12 completed the trial. Age-matched and sex-matched healthy controls provided skin and blood for proteomic and transcriptomic studies. The primary outcome was observed at 24 weeks, and additional follow-up occurred at 36 weeks. Guselkumab is a fully human immunoglobulin G1 λ monoclonal antibody that selectively binds and inhibits the p19 subunit of IL-23. Subcutaneous injections were given at the US Food and Drug Administration-approved dosing schedule for psoriasis over a 24-week period. The primary outcome was the mean change in the Psoriasis Area Severity Index (PASI) score at week 24. Secondary outcomes included pruritus, Dermatology Life Quality Index score, clinical response at week 36, and association with transcriptomics and proteomics expression. A per-protocol analysis was performed for the cohort of 4 female and 8 male patients who had a mean (SD) age of 56.5 (18.7) years. The mean improvement in PASI score, pruritus, and Dermatology Life Quality Index score was 61.8% (P < .001), 62.3% (P = .001), and 60.2% (P < .001), respectively. Nine participants (75%) achieved a 50% improvement in PASI. Among these clinical responders, at week 36, 8 of 9 achieved PASI75, and 6 of 9 achieved PASI90. No participants had pathogenic CARD14 gene variations. There was 1 serious adverse event that was not associated with the study drug. Proteomics and gene expression profiles identified dysregulation of a predominance of inflammatory pathways (such as T helper 17 and nuclear factor κ B) in participants with PRP who later responded well to treatment with guselkumab and stronger dysregulation of keratinocyte development pathways in individuals who did not respond to guselkumab. The results of this nonrandomized trial suggest that guselkumab has efficacy in treating refractory moderate to severe adult PRP. ClinicalTrials.gov Identifier: NCT03975153.

Biologics for Treatment of Pityriasis Rubra Pilaris: A Literature Review.

To describe the published efficacy and adverse event rates associated with existing biologics for the treatment of pityriasis rubra pilaris (PRP). A literature review using the PubMed database (January 1990-July 2023) was conducted. Multiple search combinations were conducted using "pityriasis rubra pilaris" and various biologics as keywords to identify relevant articles. Inclusion criteria included all study types that were published within the past 30 years in English and mentioned at least one biologic and PRP. A preliminary search yielded a total of 499 results. After screening using inclusion and exclusion criteria, 77 relevant articles (69 case reports, 5 case series, 2 clinical trials, and 1 retrospective analysis) were analyzed. TNF-α inhibitors have been evaluated and are effective in treating PRP. However, recent treatment with anti-interleukin (IL)-17 and anti-IL-23 therapies such as ustekinumab, secukinumab, and ixekizumab are emerging as new treatment options with a mean improvement in PRP Area and Severity Index scores, change in severity of erythema, scaling, and thickness of PRP lesions. From initial clinical trials, secukinumab and ixekizumab are promising treatment options for achieving remission. This review compares the efficacy for numerous biologics and a discussion to guide clinicians on benefits and risks in choosing a biologic for PRP patients. Biologics may be a favourable treatment option leading to greater patient adherence due to reduced dosing frequencies, improvement in quality of life, and reduction in frequency and severity of flares.

Rapid response of erythrodermic pityriasis rubra pilaris to tildrakizumab.

The cases of two patients with erythrodermic pityriasis rubra pilaris that showed a rapid and virtually complete response to tildrakizumab are presented. Treatment of pityriasis rubra pilaris remains challenging and there is very little evidence about use of interleukin-23 blockers in this condition. These cases may contribute to extending the available therapeutic options, especially when a fast clearance of the lesions is needed.

Successful Treatment of Pityriasis Rubra Pilaris with Risankizumab in Children.

Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disease that affects men and women of all ages, including children. PRP is characterized by follicular and palmoplantar hyperkeratosis and salmon-colored scaling plaques. The exact pathogenesis of PRP is still unknown; most PRP cases are acquired, but some cases may show a familial occurrence, often associated with a mutation in the CARD14 gene. Due to the rarity of PRP, treatment recommendations are based mainly on case reports, small case series and expert opinions and still represent a major therapeutic challenge, especially in children. A growing number of reports on treatment with biologicals, particularly anti-TNFα, has been published. However, an involvement of the IL-23/Th17 axis in both psoriasis and PRP pathogenesis may suggest that this pathway may be a potential therapeutic target. Here, we present three pediatric patients with PRP successfully treated with risankizumab. All patients exhibited a severe course of PRP and lack of response to conventional therapy, including acitretin, cyclosporine and phototherapy. A single dose of 75 mg risankizumab resulted in almost complete clearance of skin lesions in case 1 and 2 at week 4. In patient 3, clear skin was achieved after the second administration of risankizumab (150 mg). All patients continue the treatment with risankizumab, and no adverse effects have been reported up to the present time. Our study demonstrates that risankizumab, an IL-23 blocker, shows good efficacy and safety among pediatric patients with PRP.

The Effectiveness of Anti-Interleukin-17A Treatment for Pityriasis Rubra Pilaris: A Systematic Review.

Anti-interleukin-17A (anti-IL-17A) therapy has been increasingly employed as a treatment option for pityriasis rubra pilaris (PRP). In this study, we reviewed all available studies on this topic in the literature to evaluate the efficacy and safety of anti-IL-17A. Our main objective was to assess the current evidence on the efficacy and safety of anti-IL-17A therapy in the management of PRP. We searched for relevant articles on PubMed, MEDLINE, Ovid, Embase, and the Web of Science electronic databases frominception until 2022. Our inclusion criteria were as follows: randomized controlled trials (RCTs), quasi-randomized trials, or prospective observational studies that include PRP patients treated with biological treatments; studies that report clinical outcomes; and studies that compare the treatment modalities, including anti-IL-17, in the English language. A total of 19 articles involving 77 cases were reviewed afterapplying the inclusion criteria and removing duplicates. We found that type 1 PRP was the most common condition irrespective of gender, and the trunk was the most affected area. The study showed that IL-17 inhibitors had a significant impact on the patients. However, higher-level studies are required to further evaluate the therapeutic and safety effects of the treatment.

A Case Report on Pityriasis Rubra Pilaris (PRP) and Multisystem Inflammatory Syndrome in Children (MIS-C)

Pityriasis rubra pilaris (PRP) is a rare inflammatory papulosquamous skin disease of unknown etiology. There are six distinct types of PRP. A higher incidence was reported within the pediatric group, but treatment remains largely empiric, based on case reports and series. The condition is often refractory to treatment, especially initially, with topical corticosteroids and oral Acitretin the more commonly employed agents. biologic agents approved for psoriasis have been used with good effect in PRP. Multisystem Inflammatory Syndrome in Children (MIS-C) is a new disease entity that evolved as a result of the coronavirus disease 2019 (COVID-19) pandemic. Due to our incomplete understanding of the occurrence of MISC, new diagnostic and therapeutic issues have emerged. The American college of rheumatology (ACR) has recommended the use of intravenous immunoglobulin (IV IG) and/or high-dose corticosteroids as first-line treatment in these patients. In some cases of severe patients may receive such as a second dose of IV IG, Anakinra or Infliximab and Aspirin. Here we report the case of a 4-year-old boy presented with rashes. We describe the successful use of IV IG, corticosteroids, and Aspirin for treatment of MIS-C and PRP. Keywords: Pityriasis rubra pilaris, MIS-C, IV IG

411 Keratinocytes drive a specific inflammatory gene signature in pityriasis rubra pilaris

Pityriasis rubra pilaris (PRP) represents a group of rare inflammatory skin disorders. Diagnosis is often challenging, its treatment is mainly empirical and suffers from the lack of controlled trials. PRP shares overlapping clinical and histological features with psoriasis, suggesting a common underlying pathophysiology. Based on this resemblance, a role of the IL23–TH17-axis in PRP was proposed, and several cases of successful treatment of PRP with psoriasis biologics were reported. However, efficacy remains variable and these therapeutic options lack a valid immunopathological rationale.

Clinical trial of secukinumab for the treatment of pityriasis rubra pilaris with an analysis of genetic changes

Abstract Linked Article: Boudreaux et al. Br J Dermatol 2022; 187:650–658.

A Case Study Over Pityriasis Rubra Pilaris Through Ayurvedic Management

Skin disorder are common manifestation in present era and more so frequent in elder age. The patient of skin disease is more prone to experience physical, emotional and socio-economic embarrassment in the society due to disfigured appearance. Normally 10-15% of the general practitioners encounter with skin disorder in there day to day practice. Vaipadeeka and Charma kustha is a type of Kshudra kustha occurring mainly due to the imbalance of Vata and Kapha doshas resembling the Pityriasis Rubra Pilaris in its clinical manifestation. Pityriasis rubra pilaris is rare chronic disorder that causes hyper keratotic yellowing of the skin including trunk, extremities and particularly palm and sole. The palm and sole become thickened smooth and yellow fissure are common. Systemic corticosteroid, oral retinoid, immunosuppressive such as cyclosporine are the mainstay of treatment of Pityriasis rubra pilaris. The modern treatment modalities are not devoid from grave complication mainly in long term use. Owning these reasons, there is need for treatment having good efficacy and no toxic profile. So this case study was conducted considered ptyriasis rubra pilaris as Charma kustha and Vaipadeeka. Ayurvedic management was planned accordingly. Good result was witness by Virechan and shaman.

Successful Treatment of Severe Pityriasis Rubra Pilaris with Cyclosporine A in An Infant

Abstract Introduction: Pityriasis rubra pilaris (PRP) is a rare chronic papulosquamous disease and is often difficult to treat, especially in children. Here we reported a PRP infant successfully treated by cyclosporine A. Case presentation: An 18-month-old infant with severe PRP presented with reddish follicular papules and inflammatory, infiltrated erythema over the whole body. The condition rapidly and completely responded to oral cyclosporine A. Complete clearance of the lesions was achieved within 16 weeks, and no recurrence developed during 6 months of follow-up. During both treatment and follow-up, routine blood parameters, serum creatinine, serum urea nitrogen, liver function, and blood pressure were regularly monitored once a month and remained within the reference ranges. Moreover, the child grew and developed normally and the body weight increased by 3 kg. Discussion: To date, there are not well established protocols for treatment of PRP in children. Cyclosporine A has been demonstrated to be effective treatment in psoriasis in adults and children which do not respond to some other treatments. The rapid onset of action and good efficacy of cyclosporine A were observed in our case, who achieved complete response after 16 weeks of treatment without any side effects. Conclusion: Cyclosporine A might be a safe and effective option for treating severe PRP in children.

Is risankizumab effective in the treatment of pityriasis rubra pilaris?

Is risankizumab effective in the treatment of pityriasis rubra pilaris?

Treatment of Pityriasis Rubra Pilaris with Daily Low-Dose Methotrexate: A Retrospective Cohort Study

Background: Pityriasis rubra pilaris (PRP) is a rare disease that is difficult to manage. Treatment options include corticosteroids, immunomodulatory drugs, and biologics. Available literature has primarily described the use of weekly dosing of methotrexate, but there is limited data investigating the effectiveness of daily low-dose methotrexate in PRP treatment.&#x0D; Methods: A retrospective cohort study was conducted from September 2010 to December 2019 to determine the effectiveness of daily low-dose methotrexate in treating PRP.&#x0D; Results: The average duration of follow-up was 13.5 months. 14 patients were treated with oral daily low-dose methotrexate. 13 patients (92.9%) showed improvement on oral daily low-dose methotrexate. Mean time to clinical response was 5.9 weeks. In seven patients (50%), complete response on methotrexate monotherapy occurred within an average of 11.9 months. 12 patients (85.7%) developed asymptomatic transaminitis that resolved in most patients (66.7%) after dose reduction.&#x0D; Conclusions: In this study, daily low-dose methotrexate was an effective treatment of PRP and may be considered in patients unresponsive to weekly dosing. Due to the high incidence of transaminitis, the authors recommend frequent lab monitoring and screening for risk factors. Further studies are warranted to elucidate the efficacy of daily low-dose methotrexate in the management of PRP.

695 Pityriasis rubra pilaris treated with guselkumab: Interim analysis of a single-arm trial

Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disorder characterized by generalized pruritic erythematous scaly plaques and waxy palmoplantar keratoderma with painful fissuring. PRP is a life-altering condition with severe detriment to patients’ quality of life, but as of yet there is no FDA-approved medication for the treatment of PRP. The exact pathogenesis of PRP is unclear, but previous studies have demonstrated the role of the IL-23/Th17 axis in the development of this condition. We are conducting an ongoing investigator-initiated single-arm trial to investigate the use of guselkumab, a monoclonal antibody against IL-23, in the treatment of PRP (clinicaltrials.gov identifier NCT03975153). The study cohort includes patients 18-99 years of age with moderate-to-severe PRP (body surface area ≥ 10%) and no major comorbidities. Subjects received guselkumab 100 mg subcutaneous injection according to the FDA-approved dosing schedule for psoriasis (weeks 0, 4, 12, and 20) and progress was tracked via both investigator- and patient- reported outcomes. The primary endpoint of the study was at week-24; to date, six subjects have completed 24 weeks of treatment. Results from this cohort were analyzed using two-tailed paired t tests. An intention-to-treat analysis was conducted. The study cohort is composed of 4 males and 2 females with a median age of 59.5 years. Mean clinician-assessed severity as measured by PASI decreased significantly from week-0 (31.4, SD=13.5) to week-24 (10.8, SD=10.5) (p=0.0159). Five of six patients experienced a greater than 50% improvement in PASI score, and one patient did not complete the trial due to lack of improvement. Patient-reported quality of life as measured by the Dermatology Life Quality Index (DLQI) was also significantly improved from week-0 (21.3, SD=5.7) to week-24 (9.5, SD=9.4) (p=0.0074). Interim results demonstrate the disease burden of untreated PRP and the dramatic improvement in PRP symptomology following a course of guselkumab therapy.

Ptiríase rubra pilar: relato de um caso raro

OBJECTIVES: Report the case of pityriasis rubra pilaris (PRP) in a male patient at the age of 2 years and 10 months. METHODS: A review of the medical record as well as the literature regarding the present conditions, via PubMed database. RESULTS: Male patient, 2 years and 10 months, referred for the first year of appearance of pruritic erythematodescamative annular plaques in lower limbs with dissemination to the trunk, upper limbs, and face, associated with scaling in the plantar region and nail dystrophy. The biopsy presented stratum corneum parakeratotic with hypergranulosis and moderate acanthosis. The diagnosis of pityriasis rubra pilaris was considered conclusive. Isotretinoin and oral antihistamine were prescribed, having complete improvement. CONCLUSIONS: PRP remains a challenging disease, be it on its pathogeny, diagnosis or treatment. Randomized studies would aid on establishing a standardized treatment for PRP, improving the quality of life of patients suffering from this disease.

Treatment of pityriasis rubra pilaris: a case series of 28 patients.

Pityriasis rubra pilaris (PRP) is a rare chronic inflammatory dermatosis. Treatment is challenging and mostly based on scarce empirical evidence from large case series. To investigate patient characteristics and therapy outcomes in patients with PRP. In this retrospective study, patients with PRP treated at the Department of Dermatology, University Medical Center Göttingen, between 01/2005 and 10/2018, were analysed according to patient and disease characteristics and treatment experience. PRP was diagnosed in 28 patients with a mean age of 57.3 years at onset; 20 were men (71.4%) and eight women (28.6%). Of all the patients, 89.3% had comorbid diseases; metabolic disorders were particularly frequent. Seventy-eight treatment courses were identified with acitretin as the most common therapy (28.2%). Excellent response was noted in 71.4% of treatment courses with methotrexate, followed by cyclosporine (27.3%) and acitretin (19.0%). Among biologics, ustekinumab (excellent response: 75.0%) and infliximab (50.0%) were particularly effective. This study is one of the few comprehensive larger case series of patients with PRP. Acitretin may be effective, particularly for type I PRP, and other retinoids, methotrexate or cyclosporine might also be considered early in the therapeutic algorithm. Moreover, our data adds to the growing evidence that biologics are effective and safe for the treatment of PRP.

A Case of Meningococcal and HSV-2 Meningitis in a Patient Being Treated with Ustekinumab for Pityriasis Rubra Pilaris.

Pityriasis rubra pilaris (PRP) is a rare chronic inflammatory papulosquamous dermatosis affecting both adults and children. Six subtypes of PRP have been described. Recently, the management of PRP with biologic immunosuppressive agents regularly used in psoriasis has been supported by several case reports and series. Ustekinumab is an anti-IL12/23 IgG1 kappa human monoclonal antibody. It has been approved for the treatment of Crohn’s disease, plaque psoriasis, psoriatic arthritis and ulcerative colitis. It has also been reported to be effective as an off-label treatment for PRP. Current data are equivocal regarding infectious disease risk with ustekinumab administration. We describe a case of meningococcal and HSV-2 infection of the central nervous system in a patient being treated with ustekinumab for PRP.LEARNING POINTSThe administration of biologic immunosuppressive agents can result in severe life-threatening infections.Research is required on the infection potential of ustekinumab.Physicians should be aware of the possibility of infectious disease when prescribing biologic agents.Vaccination is essential in immunosuppressed adults.

Successful Treatment of Pityriasis Rubra Pilaris with Brodalumab

Pityriasis Rubra Pilaris (PRP) is a rare inflammatory skin disease with highly variable clinical appearance. Treatment of PRP remains a challenge and has been mostly guided by case reports and case series. We report the first case of pityrisis rubra pilaris that is successfully treated with combination therapy of brodalumab and methotrexate.

Use of Biologics in Pityriasis Rubra Pilaris Refractory to First-Line Systemic Therapy: A Systematic Review

Pityriasis rubra pilaris (PRP) is an uncommon, inflammatory, papulosquamous skin disease. Treatment of PRP is challenging as the disease is often refractory to conventional therapies, such as retinoids and methotrexate. There has been an increasing number of studies reporting the successful use of biologic therapy in patients with PRP; however, the data on the efficacy and safety are limited. Our objective was to evaluate the existing evidence for utilizing biologics, whether alone or in combination with established systemic therapies, in patients with treatment-resistant PRP. We systematically reviewed evidence within Medline and Pubmed databases between January 1, 2000, to March 31, 2019. Articles consisted of patients diagnosed with PRP who have failed to respond sufficiently to first-line systemic therapies, or who had comorbidities that precluded their use. In total, 363 unique articles were identified, 56 of which were considered relevant to the clinical question. Of the 56 articles highlighted, 35 met the inclusion criteria and were limited to case series and case studies. Therapy with biologics was found to be successful for both monotherapy (81.1% [27/33]) and when used in combination with existing systemic therapies (87.5% [14/16]). The existing evidence suggests that biologics may be regarded as a tool for PRP treatment alone or in combination therapy with existing treatments, although large-scale randomized clinical trials are necessary to better assess their efficacy and safety.

Treatment of pityriasis rubra pilaris type I: a systematic review.

Pityriasis rubra pilaris (PRP) is an uncommon papulosquamous inflammatory disease of the skin, which may progress to erythroderma. The diagnosis is based on both clinical and histopathological findings. There are numerous treatment options in the literature, but often reported as unsuccessful. To summarize the therapy of type I PRP in a systematic manner. We performed a systematic search following the PRISMA Guidelines based on PubMed, Web of Science, and Medline databases using the term 'pityriasis rubra pilaris treatment' (in German and English) on human subjects, published between 1997 and 2017, documenting therapy for PRP type I. A total of 449 records were identified; 148 full-text articles were assessed for eligibility and 105 articles were included in the qualitative synthesis. We identified mainly individual case reports, a few retrospective studies, and small case series. No randomized controlled trials were found. Treatment options included topical and systemic agents, and physical modalities. Based on our review, we suggest a continuous topical treatment and, when appropriate, in combination with phototherapy. As first-line therapy, we recommend a retinoid, and as second-line, a combination of retinoid and methotrexate (considering the patient's condition and side effects), azathioprine, or cyclosporine A. Biologicals can be used as third-line therapy. In case of treatment failure, biologicals can be combined with a retinoid, methotrexate, or cyclosporine A. Randomized controlled clinical trials are needed in order to provide an evidence-based high-quality standardized treatment for patients with PRP type I.

Guselkumab in the Treatment of Pityriasis Rubra Pilaris (PRP)

Guselkumab in the Treatment of Pityriasis Rubra Pilaris (PRP)