Pityriasis Rubra Research Articles

Ustekinumab: In Psoriasis and Beyond —A Derm atological Perspective

Background Ustekinumab is an interleukin 12/23 inhibitor, approved by the US-FDA for the management of moderate-to-severe plaque psoriasis and psoriatic arthropathy. Purpose This review aims to describe the dermatological implications and applications of ustekinumab. Methodology PubMed and Google Scholar were searched for scholarly articles related to ustekinumab and its utility in dermatology using the search terms “Ustekinumab” AND “Psoriasis” AND “dermatological diseases”. Studies utilising Ustekinumab in psoriasis and other dermatological indications were analysed and formulated into a systematic review discussing the utility of the drug in psoriasis, as well as other dermatological conditions. Results Ustekinumab is a valuable biologic agent for the management of psoriasis and psoriatic arthropathy, as well as other dermatological disorders like hidradenitis suppurativa, lichen planus, pityriasis rubra pilaris, Behcet’s disease, lupus erythematosus, alopecia areata and pyoderma gangrenosum. Conclusion Ustekinumab’s usage is not limited to psoriasis. Its benefit extends to many more dermatological conditions. It is considered to be the biologic of choice in childhood psoriasis and adult psoriatic patients with concurrent Crohn’s disease. Besides, it has an acceptable safety profile.

Paraneoplastic dermatosis in pediatric and adult patients

Clinical, morpho-immunological and cytogenetic characteristics of malignancies are very polymorphic. And no less heterogeneous are paraneoplastic skin presentations arising before malignant tumour manifestation and/or proceeding in parallel. In the current literature review the most common paraneoplastic dermatosis are presented: pyoderma gangrenosa, dermatomyositis, paraneoplastic pemphigus, pityriasis rubra pilaris, Bazex syndrome, necrolytic migratory erythema, ptyriasis rotunda, Sweet syndrome. It is provided modern concepts in pathogenesis, clinical features and treatment approaches.

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.

Pityriasis Rubra Pilaris refractory to ustekinumab successfully treated with guselkumab

AbstractPityriasis Rubra Pilaris (PRP) is a rare papulosquamous inflammatory dermatosis with variable clinical presentation and poorly understood pathogenesis that often poses diagnostic and therapeutic challenges. Data on currently available treatment options are limited, particularly when facing refractory cases. Both ustekinumab, an interleukin‐12 (IL‐12) and IL‐23 inhibitor, and guselkumab, an IL‐23p19 inhibitor, target the Th‐17 axis and are well established treatment options in moderate to severe psoriasis. However, increasing evidence, mostly derived from case reports and supported by recent understanding of the role of Th‐17 axis in PRP, also favours the use of IL‐17/IL‐23 pathway inhibitors in this disease. We present an adult female patient with long history of recalcitrant PRP refractory to multiple treatment options. Although initial improvement with ustekinumab was achieved, late disease progression ensued, leading to transition to guselkumab with remarkable clinical response.

Diseases categorized as autoinflammatory keratinization diseases (AiKDs), and their pathologies and treatments.

Whole-exome and whole-genome sequencing have become widespread in approximately the last 15 years, and the predisposing factors and pathomechanisms of inflammatory keratinization diseases, which have been unknown for a long time, have gradually been revealed. Hence, various inflammatory keratinization diseases are recognized to cause innate immunity hyperactivation. Therefore, we have been advocating for the clinical entity, "autoinflammatory keratinization diseases (AiKDs)" since 2017. AiKDs are inflammatory keratinization diseases caused by autoinflammatory-related pathomechanisms in the skin. The aberrant activation of innate immunity and the resultant autoinflammation in the epidermis and the superficial dermis in AiKDs cause hyperkeratosis in the epidermis. Our initially proposed concept of AiKDs included generalized pustular psoriasis and related conditions, pityriasis rubra pilaris type V, and familial keratosis lichenoides chronica. Since then, the number of diseases known to be AiKDs has increased as previously unknown disease-causing factors and pathogenetic mechanisms of inflammatory keratinization diseases have been clarified one by one. To date, porokeratosis, hidradenitis suppurative, keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK) syndrome, and AiKDs associated with epidermal growth factor receptor (EGFR) deficiency or with hepatitis and autism have been recognized as AiKDs. The concept of AiKDs is considered extremely useful in our precise understanding of the pathogeneses behind inflammatory keratinization diseases and our appropriate treatment method selection. The number of AiKDs is expected to grow with the clarification of the pathomechanisms of further inflammatory keratinization diseases.

Updates on Pityriasis Rubra Pilaris: A Scoping Review.

Pityriasis rubra pilaris (PRP) is a rare, inflammatory papulosquamous skin disease with unknown exact etiology. Historically, PRP has been challenging to diagnose, especially during the acute phase, and to treat, due to its unclear pathogenesis. To better inform clinical practice, a literature review was conducted employing a broad search strategy to capture PRP-related published studies between January 1, 2012 to October 31, 2022. Two hundred twenty-one studies were identified, which were categorized into 9 themes: (1) potential causes and triggering factors, (2) comorbidities, (3) diagnostic difficulties, (4) genetics, (5) clinical manifestations and laboratory values, (6) treatment, (7) treatment-related adverse events, (8) quality of life, and (9) other. COVID-19 infection, COVID-19 vaccination, and malignancy were the most commonly reported potential triggering factors. Misdiagnosis is very common during the early acute stages. Pathogenesis and genetic studies have further implicated caspase recruitment domain family member 14 (CARD14) mutations in the development of familial PRP (Type V) and have underlined the overlap between psoriasis and PRP. To date, there are currently no specific and validated scoring systems or tools to assess the severity of PRP. While large, randomized trials are still lacking, biologic agents remain the most effective therapy.

Dermatological manifestations of HIV: Implications for esthetic and clinical management

ABSTRACT HIV/acquired immunodeficiency syndrome has significant systemic implications, with dermatological manifestations often being the earliest and most visible indicators of infection. These skin conditions can severely impact patients’ quality of life and esthetic health. This review explores a broad range of dermatological changes associated with HIV, including HIV-specific dermatoses such as papular pruritic eruption, xerosis, eosinophilic folliculitis, prurigo nodularis, and papular mucinosis. It also covers common dermatoses such as seborrheic dermatitis, psoriasis, pityriasis rubra pilaris, and hidradenitis suppurativa. In addition, the review examines skin infections (fungal, bacterial, and viral), HIV-related malignancies (Kaposi’s sarcoma and cutaneous lymphomas), and antiretroviral therapy (ART)-related skin changes including exanthematous reactions and lipodystrophy. Special attention is given to the management of these conditions, which often involves a combination of topical and systemic treatments along with ART. The phenomenon of immune reconstitution inflammatory syndrome and its dermatological manifestations are also discussed, highlighting the complexities of managing skin reactions as the immune system recovers. Furthermore, the review addresses ectoparasitic infestations and skin complications arising from intravenous drug use, which are prevalent in HIV patients. Effective management of these dermatological issues is crucial not only for improving physical health but also for addressing the profound esthetic concerns that affect patients’ psychological well-being. Early recognition and appropriate treatment strategies can lead to better patient outcomes and enhanced quality of life. Advances in ART and dermatological therapies continue to improve the care provided to HIV patients, ensuring a comprehensive approach that considers both medical and esthetic aspects of their health. This review underscores the importance of a multidisciplinary approach in managing the diverse and complex skin conditions associated with HIV.

Tildrakizumab in Psoriasis and Beyond

Background: Tildrakizumab is an interleukin-23p19 inhibitor, approved by the Food and Drug Administration for the management of moderate-to-severe plaque psoriasis. Purpose: This review aims to describe the dermatological implications and applications of tildrakizumab. Methods: PubMed and Google Scholar were searched for scholarly articles related to tildrakizumab and its utility in dermatology using the search terms “Tildrakizumab” AND “Psoriasis” AND “other dermatological disorders.” Results: Tildrakizumab is a valuable biologic agent for the management of psoriasis. It has also been successfully used for other dermatologic disorders such as hidradenitis suppurativa, lichen planus, pityriasis rubra pilaris, lupus erythematosus tumidus, and pyoderma gangrenosum. Conclusion: Tildrakizumab’s usage is not limited to psoriasis. Its benefit extends to many more dermatologic conditions. Besides, it has an acceptable safety profile.

Pityriasis Rubra Pilaris: An Updated Review of Clinical Presentation, Etiopathogenesis, and Treatment Options.

Pityriasis rubra pilaris (PRP) is a rare papulosquamous reaction pattern with a significant impact on quality of life. Type I PRP is the most common PRP variant, presenting as erythematous papules emerging in a follicular distribution and later coalescing into plaques with characteristic islands of sparing; histologically, an alternating pattern of orthokeratosis and parakeratosis is considered the hallmark of PRP (checkerboard hyperkeratosis). Other PRP variants (types II-V) differ in their age of onset and clinical presentation. Type VI PRP is a rare PRP subtype associated with human immunodeficiency virus infection and is occasionally associated with diseases of the follicular occlusion tetrad. Caspase recruitment domain family, member 14 (CARD14)-associated papulosquamous eruption and facial discoid dermatitis are newly described disease states that have an important clinical overlap with PRP, creating shared conundrums with respect to diagnosis and treatment. The etiology inciting PRP often remains uncertain; PRP has been suggested to be associated with infection, malignancy, or drug/vaccine administration in some cases, although these are based on case reports and causality has not been established. Type V PRP is often due to inborn CARD14 mutations. Furthermore, recent literature has identified interleukin-23/T-helper-17 cell axis dysregulation to be a major mediator of PRP pathogenesis, paving the way for mechanism-directed therapy. At present, high-dose isotretinoin, ixekizumab, and secukinumab are systemic agents supported by single-arm prospective studies; numerous other agents have also been trialed for PRP, with variable success rates. Here, we discuss updates on clinical manifestations, present new insights into etiopathogenesis, and offer a survey of recently described therapeutic options.

Key Factors in the Complex and Coordinated Network of Skin Keratinization: Their Significance and Involvement in Common Skin Conditions.

The epidermis serves many vital roles, including protecting the body from external influences and healing eventual injuries. It is maintained by an incredibly complex and perfectly coordinated keratinization process. In this process, desquamation is essential for the differentiation of epidermal basal progenitor cells into enucleated corneocytes, which subsequently desquamate through programmed death. Numerous factors control keratinocyte differentiation: epidermal growth factor, transforming growth factor-α, keratinocyte growth factor, interleukins IL-1-β and IL-6, elevated vitamin A levels, and changes in Ca2+ concentration. The backbone of the keratinocyte transformation process from mitotically active basal cells into fully differentiated, enucleated corneocytes is the expression of specific proteins and the creation of a Ca2+ and pH gradient at precise locations within the epidermis. Skin keratinization disorders (histologically characterized predominantly by dyskeratosis, parakeratosis, and hyperkeratosis) may be categorized into three groups: defects in the α-helical rod pattern, defects outside the α-helical rod domain, and disorders of keratin-associated proteins. Understanding the process of keratinization is essential for the pathogenesis of many dermatological diseases because improper desquamation and epidermopoiesis/keratinization (due to genetic mutations of factors or due to immune pathological processes) can lead to various conditions (ichthyoses, palmoplantar keratodermas, psoriasis, pityriasis rubra pilaris, epidermolytic hyperkeratosis, and others).

Pityriasis Rubra Pilaris: An Uncommon Presentation

Human immunodeficiency virus (HIV) associated pityriasis rubra pilaris (PRP) or PRP Type VI is a distinctive entity reported in HIV patients. We report a patient with skin Type V (based on the Fitzpatrick scale), and HIV infection, presenting with an atypical clinical manifestation (non-acneiform), and with an uncommon distribution of this entity but characteristic histological findings of Type VI PRP. We review the clinical features, pathology and possible aetiology of this entity.

HIV: Inflammatory dermatoses

Patients living with HIV may experience a variety of inflammatory dermatoses, ranging from exacerbations of underlying conditions to those triggered by HIV infection itself. This article presents a current literature review on the etiology, diagnosis and management of atopic dermatitis, psoriasis, pityriasis rubra pilaris, lichen planus, seborrheic dermatitis, eosinophilic folliculitis, pruritic papular eruption and pruritus, in patients living with HIV.

Chronicles of A.I. Pospelov Moscow Society of Dermatovenerologists and Cosmetologists (MSDC was founded on October 4, 1891). Bulletin of the MSDC meeting N 1154

On October 17, 2023, the 1154th meeting of the Moscow Society of Dermatovenerologists and Cosmetologists named after A.I. Pospelov took place.
 The meeting was held in a face-to-face format. There were 118 participants in total. 65 applications were submitted for membership in MSDC! Due to the large number of applicants for membership in the MSDC, a list vote was held: approved unanimously.
 Two reports were presented in the clinical part of the meeting: Pityriasis rubra pilaris after suffering COVID-19 (Sechenov University); A clinical case of a rare hereditary disease ― erythrokeratoderma (State Scientific Center of Dermatovenereology and Cosmetology).
 Reports were made in the scientific part of the meeting: Topical therapy for xerosis of various genesis (Sechenov University); Modern paradigms for improving the effectiveness of treatment of allergodermatoses taking into account violations of the skin microbiome (Federal Scientific and Clinical Center of Intensive Care and Rehabilitation); The use of transcranial electrical stimulation in patients with atopic dermatitis (Sechenov University).

Case report: Area of focus of management of severe pityriasis rubra pilaris by dose optimization of adalimumab biosimilar in combination with acitretin and montelukast

Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disorder characterized by hyperkeratotic follicular papules, orange-red scaling plaques with islands of sparing and palmoplantar keratoderma. While spontaneous resolution occurs in some cases, treatment can be challenging for others. The use of biologics in PRP management has gained attention in recent studies, although their high costs and potential side effects present limitations. We present a case of a 71-year-old patient with treatment-resistant PRP who showed significant improvement through optimized adalimumab treatment. Considering the emerging role of phospholipase A2 in PRP pathogenesis, montelukast was added, further enhancing the therapeutic response. By maintaining montelukast and prolonging the adalimumab interval to 3 and 4 weeks, effective dose optimization was achieved without PRP relapse. This case report highlights the potential for adalimumab dose optimization by shortening the initial treatment interval for increased effectiveness and lengthening the interval during the maintenance phase to conserve medication doses. Montelukast appears to assist in sustaining clinical outcomes during interval prolongation, necessitating further investigation through additional studies.

HIV-associated “Pityriasis Rubra Pilaris” type 6: First case report from Turkey

A 22-year-old newly diagnosed HIV male, homosexual, presented with rash and thin scaly peeling on bilateral knees and elbows. There were multiple orange-brown scaly plaques on the extensor surfaces of his extremities. Histopathologic examination was consistent with the clinical diagnosis of Pityriasis Rubra Pilaris. CD4+ T lymphocytes were 522/μl and virus load was 19 100 copies/μl. Acitretin and antiretroviral therapy was started and he responded well within 3 months.

Eosinophils in Traditionally Noneosinophil-Rich Dermatoses.

The presence or absence of tissue eosinophilia has previously aided in the diagnosis of inflammatory skin conditions. However, recent studies have elucidated the presence of eosinophils in traditionally eosinophil-poor inflammatory skin diseases, such as dermatomyositis (DM), psoriasis, and lichen sclerosus (LS). This systematic review of the literature explores previous studies of tissue eosinophilia in skin biopsies of dermatoses that are believed to be classically poor in eosinophil. We identified 26 studies, the majority of which were retrospective reviews. The percent of specimens with increased eosinophils in psoriasis ranged from 18%-73%, pityriasis rubra pilaris (PRP) 22%-63%, LS 29%-53%, DM 15%-44%, morphea 8%-45%, hypertrophic lichen planus (LP) 0%-21%, and oral LP 0%-4%. These reports of tissue eosinophilia in reputed eosinophil-poor dermatologic conditions present a diagnostic pitfall and suggest that tissue eosinophilia itself should not be used to rule out a diagnosis of one of these conditions.

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.

Pityriasis rubra pilaris following administration of SARS‐CoV‐2 vaccine

Pityriasis rubra pilaris (PRP) is a rare, chronic, inflammatory dermatosis characterized by follicular, hyperkeratotic papules and palmoplantar keratoderma at any age. The exact etiology of the disease remains unknown, but it can be triggered by multiple factors and genetic backgrounds. Here, we describe a case of PRP occurring after SARS‐CoV‐2 vaccination. While the vaccination is generally safe, it should be kept in mind that PRP may be evoked by SARS‐CoV‐2 vaccination for early recognition of the cause and prognosis of the patients.

Patients with keratinization disorders due to ABCA12 variants showing pityriasis rubra pilaris phenotypes.

Pathogenic variants in ABCA12 are important causative genetic defects for autosomal recessive congenital ichthyoses (ARCI), which include congenital ichthyosiform erythroderma (CIE), harlequin ichthyosis, and lamellar ichthyosis. In addition, pathogenic variants in ABCA12 are known to cause a localized nevoid form of CIE due to recessive mosaicism. We previously reported siblings who carried an ABCA12 variant but did not show a "congenital" phenotype. They were considered to have pityriasis rubra pilaris (PRP). Here, we present a further patient with ABCA12 variants whose phenotype was not congenital ichthyosis, in an independent family. Notably, these three patients had geographic unaffected areas. Such areas are not usually found in patients with ARCI who have ABCA12 variants, suggesting mild phenotypes for these patients. Interestingly, the histological features of the ichthyotic lesions in these patients resembled those of PRP. All three patients had homozygous pathogenic missense variants in ABCA12. Our findings expand the phenotypic spectrum of patients with ABCA12 variants.

Patient-reported cutaneous signs and symptoms of adult pityriasis rubra pilaris and correlation with quality of life and clinician-reported severity: A cross-sectional study

Patient-reported cutaneous signs and symptoms of adult pityriasis rubra pilaris and correlation with quality of life and clinician-reported severity: A cross-sectional study