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.

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.

Pityriasis rubra pilaris heralding diagnosis of urothelial carcinoma: a case report.

Pityriasis rubra pilaris is a papulosquamous inflammatory dermatosis that can be associated with HIV, autoimmunity, infections, certain medications, and neoplasms. Paraneoplastic pityriasis rubra pilaris has previously been reported in association with solid organ malignancies and once with leukemia. Herein, we present an elderly man with paraneoplastic pityriasis rubra pilaris, heralding the diagnosis of low-grade papillary urothelial carcinoma. Our patient's pityriasis rubra pilaris resolved after surgical resection of the tumor.

Pityriasis rubra pilaris with erythema gyratum repens-like eruption and resolution with ustekinumab.

Erythema gyratum repens (EGR) is a rare paraneoplastic disorder often preceding the diagnosis of underlying malignancy by 9 months on average, while pityriasis rubra pilaris (PRP) is an uncommon papulosquamous inflammatory disease. We present the case of a 58-year-old woman with an EGR-like eruption transforming from resolving PRP, without associated malignancy. Her rash dramatically resolved within a month of ustekinumab initiation, which supports this presentation as a unique entity.

Early Presentation of Pityriasis Rubra Pilaris Mimicking Tinea Corporis: Diagnostic Challenges of a Rare Skin Condition.

BACKGROUND Pityriasis rubra pilaris (PRP) is a rare chronic inflammatory skin condition characterized by follicular, papulosquamous, reddish-orange scaling, palmoplantar keratoderma, and erythema with islands of sparing. Its heterogeneous clinical presentation makes the diagnosis of PRP quite challenging, especially at the initial presentation, as it can mimic common skin conditions. CASE REPORT We present a case with an early presentation of PRP in a 61-year-old Malay woman with underlying uncontrolled diabetes, and discuss evolving clinical course of her disease. She presented to a primary care clinic with a 3-week history of itchy, ring-like skin lesions that started on her neck and chest but subsequently spread widely on her chest, back, and upper extremities. She was first treated as having extensive tinea corporis but responded poorly to multiple courses of antifungal treatment. An initial skin biopsy that was taken at the dermatology clinic revealed features suggestive of erythema annulare centrifugum. However, despite topical steroid treatment, her skin condition evolved further and she developed generalized erythroderma along with follicular hyperkeratosis and palmoplantar keratoderma. A repeat biopsy finally confirmed the diagnosis of PRP. CONCLUSIONS Making the diagnosis of PRP is challenging for clinicians. However, clinicians should approach any common skin problem that does not respond to treatment appropriately, with consideration of other uncommon skin disorders. A repeat skin biopsy may be considered if there are any doubts about the diagnosis. A clinical and histopathological correlation is important to aid in the diagnosis of PRP.

Paraneoplastic Pityriasis Rubra Pilaris Preceding Leukemia.

ABSTRACT: Pityriasis rubra pilaris (PRP) is a rare, chronic papulosquamous disorder that presents with scaling plaques, palmoplantar keratoderma, and keratotic follicular papules. Typically, there are distinctive unaffected areas referred to as "islands of sparing." Pityriasis rubra pilaris has been associated with various immunodeficient states and malignancies.The authors conducted a literature review using MEDLINE, PubMed, and Google Scholar, documenting all known cases of PRP associated with malignancy; 15 cases were found in the literature. They also present the case of a 49-year-old White man who, prior to referral to dermatology, was seen in urgent care for widespread pruritic rash. Physical examination in the dermatology clinic revealed confluent, scaly erythematous papules coalescing into plaques with island of sparing involving the trunk and upper and lower extremities. Bilateral palms and soles showed hyperkeratosis with fissuring. He was diagnosed with PRP after punch biopsy and began a new course of topical corticosteroid therapy. Hematology was consulted because of abnormal complete blood count results, and he was subsequently diagnosed with chronic lymphoid leukemia.Treatment of PRP is largely based on clinical experience and may involve corticosteroids, immunomodulators, or biologic therapy. The relationship between PRP and malignancy is unknown. Current theories postulate it may be driven by tumor production of functional peptides or antigen cross-reactivity between cancer cells and the skin. This is the second reported case of PRP as a manifestation of leukemia, and the first of chronic lymphoid leukemia. Although not yet understood, the documented relationship between PRP and malignancy prompts screening for cancer in all patients with new-onset PRP.

An old entity, a new trigger: Post COVID-19 vaccine pityriasis rubra pilaris.

BACKGROUND: Pityriasis rubra pilaris (PRP) is a rare, acquired, chronic papulosquamous dermatosis which can occur in all ages. PRP can be associated with infection, autoimmunity, drugs and malignancies, and can be idiopathic. OBJECTIVE: PRP following vaccination has been rarely described in the literature. To the best of our knowledge, we report the first case of PRP two weeks following COVID-19 vaccination (Covishield). CASE REPORT: A 72-year-old male presented to the outpatient dermatology department at All India Institute of Medical Sciences - Bhopal with minimally pruritic superficial plaques since one week. The patient was vaccinated against COVID-19 with Covishield two weeks earlier. The lesions developed as erythematous scaly follicular papules and plaques over axilla that rapidly spread to the trunk in the following weeks and involved palms and soles as well as thickening and fissuring. The clinical features suggested PRP. The histopathology showed epidermal acanthosis with hypergranulosis alternating with parakeratosis and orthokeratosis with broad rete ridges with follicular plugging. The patient had started taking topical corticosteroids and emollients, which proved effective. There was no recurrence after receiving a second dose on follow-up. CONCLUSION: In patients presenting with new onset PRP in this COVID-19 era, the possibility of vaccine as a trigger should be taken into consideration, and further dosing should be carefully monitored in view of possible recurrence.

Pityriasis rubra pilaris post-infection due COVID-19: case report / Pitiriasis rubra pilaris post-infección por COVID-19: reporte de un caso

Abstract Case description: 32-month-old boy, IgG positive for SARS-CoV-2, presented to the emergency department with dermatologic lesions. Clinical findings: Four days before admission, he presented skin eruptions with redness and pruritus on hands and feet. Generalized papular erythema was evidenced, upper extremities with diffuse erythematosquamous plaques, palmoplantar keratoderma, so he was evaluated by a dermatologist who diagnosed pityriasis rubra pilaris. Treatment and outcome: rehydrating cream, cetirizine 0.5 mg/kg/day every two days, and prednisolone 2 mg/kg/day in the morning. He was discharged after 14 days, the patient presented clinical improvement, but the erythematous lesion persisted on the trunk and extremities. In the evaluation, after three months, the patient did not show the described lesions, evidencing an improvement and clinical resolution of the dermatological problems. Clinical relevance: We report a patient with pityriasis rubra piloris associated with a post-infection by SARS-CoV-2 that had not been described before.

Resumen Descripción del caso: Niño 32 meses de vida, con IgG positivo para SARS-CoV-2, acude al servicio de emergencia por presentar lesiones dermatológicas. Hallazgos clínicos: Cuatro días antes del ingreso presentó erupciones en la piel, con enrojecimiento y prurito en manos y pies. Se evidenció eritema papular generalizado, extremidades superiores con placas eritematoescamosas difusas, queratodermia palmo-plantar por lo que es evaluado por dermatólogo quien diagnostica pitiriasis rubra pilaris. Tratamiento y resultado: Crema rehidratantes, cetirizina 0.5 mg/kg/día cada 2 días y prednisolona 2 mg/kg/día por la mañana. Fue dado de alta a los 14 días, el paciente presenta mejora clínica, pero aún persiste la lesión eritematosa en tronco y extremidades. En la evaluación a los tres meses el paciente no mostró las lesiones descritas, evidenciando una mejoría y resolución clínica de los problemas dermatológicos. Relevancia clínica: Se reporta un paciente con afectación por pitiriasis rubra piloris asociado a una post-infección por SARS-CoV-2 que no se había descrito antes.

Secukinumab monotherapy successfully treated severe refractory type V (atypical juvenile) pityriasis rubra pilaris: A case report and literature review.

Pityriasis rubra pilaris (PRP) is a rare heterogeneous group of papulosquamous inflammatory disorders with unknown etiology. PRP is often resistant to many conventional therapies which has made more challenging on treatment. More recently, several studies have shown encouraging clinical results of secukinumab in the treatment of PRP in adult, but no studies have explored its effects in children. We herein report a 7-year-old boy with severe type V PRP responded rapidly to secukinumab monotherapy (150 mg once weekly) when conventional therapies have failed. The patient showed rapid and dramatic improvement of erythema, palmoplantar hyperkeratosis, scaling, and itching within only 5 weeks, with no adverse effects. Secukinumab could be considered as a treatment option for refractory PRP in children, as recently reported in adult.

What's New in Genetic Skin Diseases.

The discoveries of new genes underlying genetic skin diseases have occurred at a rapid pace, supported by advances in DNA sequencing technologies. These discoveries have translated to an improved understanding of disease mechanisms at a molecular level and identified new therapeutic options based on molecular targets. This article highlights just a few of these recent discoveries for a diverse group of skin diseases, including tuberous sclerosis complex, ichthyoses, overgrowth syndromes, interferonopathies, and basal cell nevus syndrome, and how this has translated into novel targeted therapies and improved patient care.

A Review on Pityriasis Rubra Pilaris.

Pityriasis rubra pilaris (PRP) is an idiopathic, papulosquamous inflammatory dermatosis. It is characterized by hyperkeratotic follicular papules coalescing into orange-red scaly plaques, islands of sparing, and palmoplantar keratoderma. PRP can be subdivided into six clinical subtypes according to Griffiths' classification, based on age of onset, disease extent, prognosis, and other associated features. The sixth subtype of PRP occurs in individuals affected by HIV infection, and retroviral screening in all de novo cases of PRP is advised. Other reported associations include various infections, autoimmunity, drugs, and malignancies, although the true significance of these is still unclear. The genetic basis for familial cases, most commonly categorized under the fifth subtype, has been mapped to gain of function mutations in the caspase recruitment domain family, member 14 (CARD14) gene. Treatment of PRP remains a challenge to this day due to a paucity of high-quality evidence. Therapeutic regimens have been guided mostly by case reports and case series, with the mainstay of treatment being oral retinoids. Recently, biologics have emerged as a promising treatment for PRP. We present a review of the clinicopathologic features, pathogenesis, associated disorders, and treatment of PRP, with an emphasis and critical appraisal of the existing literature on the latter.

[Pityriasis rubra pilaris]. / Pityriasis rubra pilaire.

Pityriasis rubra pilaris is a rare heterogeneous dermatosis associating three clinical signs to different degrees: follicular corneal papules, reddish-orange palmoplantar keratoderma and erythematosquamous lesions that may in some cases be very extensive, interspersed with patches of healthy skin. The aetiology is unclear, and in most cases, the trigger factors consist of trauma or infection, probably in subjects with an existing predisposition. In other cases, the condition is associated with immunological disorders or, in familial cases, genetic keratinisation abnormalities similar to ichthyosis. Given the widely varying signs, several classifications have been proposed, based on clinical criteria and outcomes. The outcome varies in accordance with the clinical forms involved. Therapeutic approaches are poorly qualified and there have been no clinical trials due to the rarity of the disease. However, the best results appear to have been obtained using oral retinoids, with second-line therapy comprising methotrexate and cyclosporine. The landscape of therapeutic strategy seems to be changing with the advent of new anti-tumour necrosis factor and anti-interleukin-12/23 antibodies.

Methotrexate treatment in a case of juvenile pityriasis rubra pilaris.

An 8-year-old boy who was initially diagnosed with plaque psoriasis failed management with topical therapies and skin biopsy confirmed the suspected diagnosis of juvenile pityriasis rubra pilaris (PRP). Pityriasis rubra pilaris is a rare inflammatory disorder of the skin characterized by follicular keratotic papules coalescing into plaques, along with palmoplantar keratoderma. Treatment modalities include topical and systemic therapies, although previous studies have not shown much benefit with methotrexate in children. We present a case in which methotrexate led to significant improvement of the skin findings in a child with type IV juvenile pityriasis rubra pilaris.