Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients.

INTRODUCTION: PRP is a rare entity of unknown etiopathogenesis. Lack of clinical practice guidelines makes management challenging for clinicians. OBJECTIVE: To add our experience to the corpus evidence on PRP. METHODS: This was a retrospective, descriptive, and multicentric study of 65 patients with PRP, the largest European case series of patients with PRP ever reported. RESULTS: PRP was more prevalent in men with a mean age of 51 years, yet erythrodermic forms presented in older patients (mean age, 61 years).Six (75%) pediatric patients and 10 (60%) non-erythrodermic adults controlled their disease with topical corticosteroids. However, 26 (68%) erythrodermic patients required biologic therapy as the last and effective therapy for a mean 6.5 months to achieve complete response. CONCLUSION: Our study showed a statistical difference in terms of outcome and response to treatment between children, or patients with limited disease and patients who develop erythroderma.

Post-infectious pityriasis rubra pilaris in pediatric patients. / Pitiriasis rubra pilaris posinfecciosa en pacientes pediátricos.

Pityriasis rubra pilaris (PRP) is a rare skin condition. The etiology of PRP is unknown; however, it has been associated with infections, autoimmune diseases, and neoplasms. Here we describe the cases of 2 pediatric patients with PRP triggered by a respiratory syncytial virus infection concurrently with obstructive bronchial syndrome. PRP resolved after treatment with topical emollients, topical corticosteroids, and calcineurin inhibitors.

La pitiriasis rubra pilaris (PRP) es una enfermedad dermatológica poco frecuente. Se desconoce su etiología, sin embargo, se ha asociado a infecciones, enfermedades autoinmunes y neoplasias. Se describen los casos de dos pacientes pediátricos que presentaron PRP gatillada por una infección por virus sincicial respiratorio mientras cursaban un síndrome bronquial obstructivo. Los cuadros de PRP remitieron luego del tratamiento tópico con emolientes, corticoesteroides tópicos e inhibidores de la calcineurina.

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.

Two Incidental Sibling Diagnoses of Netherton Syndrome in Separate Visits: A Case Report.

Netherton syndrome (NTS) is a genetic disorder that predominantly affects the hair and the skin, and it can have a wide variety of presentations. The genetic syndrome is more common with consanguineous parents. Given the rarity and varying presentation of the condition, a few cases have been reported in the literature. We present an unusual case of two incidental diagnoses of NTS in siblings of consanguineous parents, manifesting as erythroderma and other symptoms that were initially diagnosed as pityriasis rubra pilaris and psoriasis in separate visits. Physicians must maintain a high index of suspicion when faced with chronic skin conditions and hair shaft abnormalities that may have been present since childhood to avoid the sequela of inadvertent prolonged or misdiagnosis.

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.

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

OBJECTIVE: To describe the published efficacy and adverse event rates associated with existing biologics for the treatment of pityriasis rubra pilaris (PRP). DATA SOURCES: 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. STUDY SELECTION AND DATA EXTRACTION: 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. DATA SYNTHESIS: 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. RELEVANCE TO PATIENT CARE AND CLINICAL PRACTICE: 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. CONCLUSIONS: 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.

Resolution of Pityriasis rubra pilaris induced ectropion with oral dexamethasone. A case report.

An 82-year-old male presented with generalised Pityriasis rubra pilaris (PRP) managed initially by dermatology team. The patient did not respond to first- and second-line treatment, including oral acitretin, steroid creams, and methotrexate, and developed bilateral cicatricial ectropion, for which he was referred to oculoplastic team for surgical management. A head injury resulting in subacute subdural haematoma, managed with a week course of low dose oral dexamethasone, resulted in the improvement of his skin condition and complete resolution of the cicatricial ectropion within a few weeks. Thus, systemic treatment of PRP with oral dexamethasone may be considered sooner in the treatment of cicatricial ectropion in similar cases.

Delayed Presentation of Pityriasis Rubra Pilaris in a Patient on Treatment With Ponatinib.

Pityriasis rubra pilaris (PRP) is a rare dermatologic condition whose etiology is largely unknown. However, some medications, including ponatinib, have been implicated. Our case features an 80-year-old patient who developed PRP after two-and-a-half years of ponatinib use. We present this case due to the rare presentation of ponatinib-induced PRP as well as its significantly delayed presentation.

Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients.

INTRODUCTION: PRP is a rare entity of unknown etiopathogenesis. Lack of management guidelines makes it a challenge for clinicians. OBJECTIVE: To add our experience to increase evidence about PRP. METHODS: We performed a retrospective, descriptive and multicentric study of 65 patients with PRP, being the largest European case series of patients with PRP. RESULTS: PRP was more frequent in male patients with an average age of 51 years, but erythrodermic forms presented in older patients (average age 61 years). Six (75%) paediatric patients and ten (60%) non-erythrodermic adults controlled their disease with topical corticosteroids. On the contrary, 26 (68%) erythrodermic patients required biologic therapy as last and effective therapy line requiring an average of 6.5 months to achieve complete response. CONCLUSION: Our study showed a statistical difference in terms of outcome and response to treatment between children or patients with limited disease and patients who develop erythroderma.

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.

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.

Utility of Dermoscopy in the Diagnosis of Erythroderma: A Cross-Sectional Study.

Background: It is difficult to diagnose the underlying cause of erythroderma on mere clinical presentation. The role of dermoscopy in diagnosing erythroderma secondary to various etiologies is evolving. Aim and Objectives: This study aimed to observe the dermoscopic features of erythroderma secondary to different cutaneous disorders and compare them with clinical features and histopathology. Materials and Methods: Twenty-nine consecutive patients of erythroderma were enrolled in the study. Dermoscopy was performed on every case using a Heine Delta II Dermatoscope with 10x magnification in polarized mode. A histopathological examination was conducted to confirm the diagnosis. Results: Eight patients were diagnosed with psoriasis, five with endogenous eczema, four with pityriasis rubra pilaris (PRP), three with pustular psoriasis, two with drug rash secondary to antitubercular therapy, two with dermatophytic infection, one patient each of atopic dermatitis, crusted scabies, pemphigus foliaceous, drug reaction with eosinophilia and systemic symptoms, and mycosis fungoides. Characteristic dermoscopic features were observed in erythroderma due to psoriasis, PRP, pustular psoriasis, endogenous eczema, scabies, and dermatophytosis. Differentiation of other disorders based on dermoscopy alone was difficult, and clinico-histopathological correlation was crucial to reach a diagnosis. Conclusion: Dermoscopic features of classical patterns of skin disorders are preserved even in the corresponding erythrodermic or unstable stage. Dermoscopic features of erythroderma secondary to psoriasis, pustular psoriasis, PRP, endogenous eczema, scabies, and dermatophytosis are clearly differentiating, whereas the dermoscopic features in other causes of erythroderma are overlapping. Thus, dermoscopy can be a good screening tool in the clinical assessment of erythroderma.

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.