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.

Pitiriasis rubra pilaris tratada con lámpara excímero / Pityriasis rubra pilaris treated with excimer lamp

Resumen La pitiriasis rubra pilaris, es una dermatosis inflamatoria papuloescamosa e hiperqueratósica de origen desconocido y de progresión crónica, la cual puede evolucionar incluso a eritrodermia. El presente caso trata de un paciente de 27 años portador del virus de inmunodeficiencia humana, diagnosticado con pitiriasis rubra pilaris tipo IV, inicialmente tratado con corticosteroide tópico y fototerapia, por cuatro meses. Sin embargo, presentó reactivación de las lesiones, por lo que se recurrió a la aplicación de lámpara excímero, utilizada en otras patologías dermatológicas, mas no de uso habitual en la pitiriasis rubra pilaris.

Abstract Pityriasis Rubra Pilaris is an inflammatory papulosquamous and hyperkeratic dermatosis of unknown cause and chronic progression which can envolve even into erythroderma. This case deals with a 27-year old male patient carrier of VIH who was diagnosed with PRP type IV. Initially, it was treated with topical corticosteroid and phototherapy for four months. However, it showed reactivation of the injuries; therefore, excimer lamp was employed, which is used in other dermatologic pathologies but it is not a regular treatment for PRP type IV.

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: une dermatose mal connue.

Pityriasis rubra pilaris is a rare heterogeneous disorder characterized by follicular keratosis, perifollicular erythema and palmoplantar hyperkeratosis. The aetiology is still unknown. In the majority of cases some triggering factors are found such as trauma or bacterial infection, possibly on a predisposed condition. In other cases, some immunological disorders are associated, and in familial cases a genetic disorder of keratinization has been suggested. The evolution is variable according to the clinical type. The treatment is not well defined, and there is a lack of clinical trials. The best results however are obtained with oral retinoids, methotrexate or ciclosporine as alternative therapy. New TNF inhibitors and anti-IL-12/23 showed a good result and could be have interest in the future.

Epidemiologic, Clinicopathologic, Diagnostic, and Management Challenges of Pityriasis Rubra Pilaris: A Case Series of 100 Patients.

IMPORTANCE: Pityriasis rubra pilaris (PRP) is a rare papulosquamous disorder with limited epidemiologic and clinicopathologic data. Little information is available on long-term outcomes, comorbidities, and treatment efficacy. OBJECTIVE: To evaluate objective and subjective disease experience metrics from the perspectives of patients and clinicians. DESIGN, SETTING, AND PARTICIPANTS: One hundred patients with a putative diagnosis of PRP and who elected to participate completed a comprehensive survey, followed by acquisition of their medical records, including histopathology slides and reports. The data were analyzed separately from the health care clinician and the patient perspectives. Two academic dermatologists examined clinical notes, pathology reports, and photographs, confirming diagnoses via predetermined criteria. Patients were categorized into 4 levels of diagnostic certainty to allow stratification of the findings for subgroup analysis. Patients with a diagnosis of PRP were solicited through patient support organization websites. MAIN OUTCOMES AND MEASURES: Clinical outcomes, unexpected association of comorbidities, and efficacy (or lack of it) of various treatment modalities. RESULTS: Among the 100 patients, 50 were diagnosed as having classic, unquestionable PRP. The patients were a median of 61 years old (range, 5-87 years), and 46% were female. Fifty were categorized as level 1 diagnostic certainty, 15 as level 2, 30 as level 3, and 5 as level 4. Of the level 1 patients, 13 (26%) were correctly diagnosed at initial presentation; diagnosis was delayed, on average, by 29 months (range, 0.25-288 months; median, 2 months); and 27 (54%) having undergone 2 or more biopsies. At enrollment, PRP symptoms had persisted in 36 patients (72%) for an average of 58 months (range, 1-300 months; median, 30 months). Thirty-one patients (62%) had comorbidities, including hypothyroidism (20%). Nearly all patients (98%) received some form of therapy. Patients cited topical emollients, corticosteroids, and salicylic acid along with oral retinoids, methotrexate, and tumor necrosis factor inhibitors as most helpful. CONCLUSIONS AND RELEVANCE: Pityriasis rubra pilaris remains a challenging diagnosis without established and specific treatment. Our data highlight new potential avenues for research with therapeutic perspective.

A review of the diagnosis and management of erythroderma (generalized red skin).

Erythroderma is a condition caused by several etiologies that result in red inflamed skin on 90% or more of the body surface. To optimize the diagnosis and management of the erythrodermic patient, healthcare professionals should be familiar with the underlying etiologies and treatment modalities. Patients with erythroderma require immediate attention as they may face a variety of medical complications. Early detection and effective management of these complications significantly reduce mortality and morbidity of this potential dermatologic emergency. This review highlights the underlying common diagnoses, assessment, and management of the patient with erythroderma.

Paraneoplastic pityriasis rubra pilaris as the presenting manifestation of metastatic squamous cell carcinoma.

Pityriasis rubra pilaris (PRP) is a rare idiopathic papulosquamous eruption. Few cases of PRP have been reported in association with malignancies. We report a case of an 83-year-old Caucasian male who presented with recalcitrant paraneoplastic PRP as the presenting manifestation of metastatic squamous cell carcinoma with unknown primary. Treatment with chemotherapy and radiation led to temporary radiologic and symptomatic regression of the cancer as well as resolution of cutaneous findings. This suggests a direct relationship between the PRP and the underlying malignancy in this patient. This case highlights a rare, but important phenomenon in which PRP may act as a harbinger for underlying malignancy.

A case of pityriasis rubra pilaris associated with membranous nephropathy.

Pityriasis rubra pilaris (PRP) is a rare idiopathic dermatosis which may be associated with autoimmune diseases, HIV infection, and internal malignancies. Its association with renal diseases is, however, much less recognized. We report a case of PRP with associated membranous nephropathy (MN), which resolved spontaneously with resolution of the dermatosis. This is only the second reported association between PRP and MN of which we are aware. Further reports of such an association will strengthen the evidence for the two conditions being linked and may thereby shed light on the pathogenesis of both PRP and MN.

Pitiriasis rubra pilaris clásica juvenil: reporte de un caso / Classic juvenile pityriasis rubra pilaris: a case report

La pitiriasis rubra es una dermatosis inflamatoria rara de causa desconocido. Se clasifica en cinco tipos basado en la edad de inicio, curso clínico, características morfológicas y pronóstico. En la población pediátrica el tipo III o clásico juvenil es el segundo en frecuencia. Se caracteriza por hiperqueratosis folicular, la que puede acompañarse de queratodermia palmoplantar y alteraciones ungueales. Se presenta el caso de un paciente varón, de nueve años de edad con pitiriasis rubra pilaris clásica juvenil en tratamiento con acitretina, con buena respuesta clínica...

Pityriasis rubra pilaris is a rare inflammatory dermatosis of unknown cause. It is classified into five types bases on age of onset, clinical course, morphology and prognosis. In pediatric population classic juvenile or type III is the second most common type. It is characterized by follicular hyperkeratosis, which may be associated with palmoplantar keratoderma and nail changes. We present the case of a 9-years-old male patient with classic juvenile pityriasis rubra pilaris successfully treated with acitretin...

Pityriasis rubra pilaris: presentación de casos / Pityriasis rubra pilaris: presentation of cases

Se realizó una presentación de casos, de dos pacientes con lesiones dermatológicas, donde existe correlación clínico-histopatológica, de Pityriasis rubra pilaris. La intención de este trabajo es que en la práctica dermatológica se piense en esta dermatosis. Para ello se tuvieron como objetivos: viabilizar la comprensión de esta entidad, contribuir a realizar el diagnóstico diferencial con otros cuadros como son dermatitis seborreica, queratodermia palmo-plantar, psoriasis, entre otros. En la dermatosis objeto de estudio, su cuadro histológico es característico, y debe introducirse alimentos en la dieta ricos en vitamina A, beta carotenos y azufre, pues el factor alimenticio juega un papel importante en aquellas afecciones donde la etiología por déficit de las vitaminas u otro oligoelemento está presente. Se revisan aspectos clínicos, asociaciones con otras enfermedades, el tratamiento de esta dermatosis. Se utilizó el método aleatorio, al azar, longitudinal, corroborando el diagnóstico por biopsia de piel de las pacientes. Las pacientes estudiados fueron: ARG, de 5 años, femenina, blanca remitida por el pediatra por lesiones en placas máculo amarillo-hipocrómicas–papulosas, distribuidas en superficie de extensión de brazos y piernas; LSG, de 19 años, femenina, blanca, que es remitida por presentar lesiones en placas máculo-hipocrómico pápulo-escamosas bordes difusos, localizadas en muslo y pierna derechos, se constata alopecia difusa del pelo del cuero cabelludo. El tratamiento local indicado fue una pomada con reductor, ácido salicílico y vaselina, la orientación alimenticia fue dirigida a ingerir alimentos ricos en vitamina A o beta carotenos, y alimentos ricos en azufre, insistiéndoles en esta parte del tratamiento por los antecedentes de ambas pacientes a la no ingestión de estos alimentos. A lo largo de 10 años hemos revisado anualmente la evolución de las pacientes, no existiendo recidivas hasta el momento, manteniendo ambas el régimen dietético orientado.

We presented the cases of two patients with dermatologic lesions, where there it is clinic- histopathologic correlation, of Pityriasis rubra pilaris. The intention of this work is taking into account this dermatosis in the dermatologic practice. To achieve that our objectives were: allowing the comprehension of this entity, contributing to the differential diagnosis with other conditions like seborrheic dermatitis, palmoplantar keratoderma, psoriasis and others. The histological picture of the studied dermatosis is characteristic, and there should be introduced in the diet foods rich in vitamin A, beta carotenes and sulfur, because the alimentary fact plays an important role in those conditions where the etiology by vitamins or other oligo-element deficit is present. We reviewed clinical aspects, the association with other diseases, the treatment of this dermatosis. We used the randomized, longitudinal method, corroborating the diagnostic by patient's skin biopsy. The studied patients were: ARG, white, female, 5-years-old patient send by the podiatrist because she had lesions in yellow macula hypochromic- papular plates, located in extension surfaces of arms and legs; LSG, white, female, 19-years-old patient send presenting lesions in macula- hypochromic papular- squamous plaques with diffuse rims, located in the right thigh and leg, stating diffuse alopecia of the scalp. The indicated treatment was an ointment with reductive, salicylic acid and vaseline. They were advised to eat foods rich in vitamin A or beta carotenes and sulfur, insisting in this part of the treatment because they had antecedents of not eating these foods. We have followed the evolution of these patients for ten years without relapses, having the patients kept the oriented dietetic regime.

Pityriasis rubra pilaris--a retrospective single center analysis over eight years.

BACKGROUND: Pityriasis rubra pilaris (PRP) is a rare papulosquamous dermatosis. We evaluated evaluate co-morbidities, complications, and outcome of treatment regimens. PATIENTS AND METHODS: This is a retrospective study at an academic teaching hospital. We analyzed all patients with the definite diagnosis of PRP seen since 2001. Epidemiologic data, co-morbidities, response to and course during treatment were investigated. RESULTS: We identified 10 PRP-patients (6 men, 4 women), mean age 56.4 years, with type I (n = 9) and type IV (n = 1). Three patients had internal co-morbidities (atrial fibrillation with cardiac insufficiency, dilated cardiomyopathy, diabetes mellitus). Two patients needed psychiatric treatment because of depression. PRP caused ectropium (2 x), diffuse effluvium (1 x), and stenosis of the outer ear canal (1 x). We did not observe a spontaneous remission. Among 9 patients with PRP type I, five were treated with acitretin (two of them as Re-PUVA), and two with methotrexate (in one patient combined with fumaric acids). Systemic corticosteroids were not effective. One patient was treated with infliximab i.v., 5 mg/kg body weight. Starting with the first application, inflammatory activity decreased and erythema got paler. The treatment was well tolerated. CONCLUSIONS: PRP type I is a severe, chronic inflammatory dermatosis responding hesitantly to classic systemic therapies. Tumor necrosis factor-alpha antagonists are an effective treatment option for difficult cases.

Pityriasis rubra pilaris: a review of diagnosis and treatment.

Pityriasis rubra pilaris (PRP) is a rare inflammatory dermatosis of unknown etiology, and finding a successful therapy can be challenging. PRP occurs equally in men and women. In some patients, associated autoimmune diseases, infections, or malignancies are possible trigger factors. PRP shows a bimodal age distribution, peaking in the first as well as in the fifth to sixth decade. Its classification into five subgroups is based on age at onset, clinical course, morphologic features, and prognosis. More than 50% of patients are best classified as type I with adult-onset PRP. This form is also characterized by high spontaneous remission rates (80%) within 1-3 years. Clinically, the classical adult (type I) and classical juvenile (type III) forms appear to be the same except for the patient's age. Recently, the designation of a new category of PRP (type VI) has been proposed that is characterized by the presence of HIV infection with different clinical features and a poorer prognosis. Typical morphologic features of PRP are erythematosquamous salmon-colored plaques with well demarcated islands of unaffected skin. Often, keratoderma of the palms and soles is present. In patients with extensive disease, ectropion is a dreaded complication. Histology shows hyperkeratosis, alternating orthokeratosis and parakeratosis in a checkerboard pattern, and focal acantholytic dyskeratosis. Descriptions and therapeutic experiences are mainly based on case reports. Mostly, systemic retinoids, methotrexate, and other immunosuppressive agents as well as UV light therapy are applied, with varying response rates. In recent years, treatment with so-called 'biologics' is becoming more and more popular for treating recalcitrant PRP. We present a review of the clinical features, histopathologic findings, classification, differential diagnoses, and treatment of PRP.

Pityriasis rubra pilaris with preceding cytomegalovirus infection.

Pityriasis rubra pilaris (PRP) is a papulosquamous disorder comprising 6 clinical types. Some factors - including abnormal vitamin A metabolism, internal malignancies, autoimmune diseases, infection and trauma - are thought to be involved in the etiology. Recently, human immunodeficiency virus (HIV)-associated PRP has been reported to have distinct clinical features, such as nodulocystic acne and lichen spinulosus alongside PRP. We report here the case of a 38-year-old female with onset of classical PRP after a high fever. Virological studies indicated that the patient had primary systemic cytomegalovirus (CMV) infection and not HIV infection. Our case suggested that primary CMV infection might have triggered typical PRP that was clinically different from HIV-associated PRP.

Pityriasis rubra pilaris, type IV.

A 4-year-old girl presented with a 3-year history of demarcated, salmon-pink, hyperkeratotic plaques, which were symmetrically distributed on the elbows, knees, ankles, and dorsal aspects of the hands and feet. A diffuse, orange-pink palmoplantar keratoderma was also evident. Clinical and histologic findings were consistent with a diagnosis of pityriasis rubra pilaris (PRP), type IV (circumscribed juvenile). Type IV PRP develops in prepubertal children, is typically localized to the distal aspects of the extremities, and has an unpredictable course. Although ultraviolet (UV) radiation can potentially exacerbate PRP, our patient has improved with broad-band UVB phototherapy.

[Pityriasis rubra pilaris]. / Pityriasis rubra pilaris.

Pityriasis rubra pilaris (PRP) is a rare papulosquamous disease with typical onset during the first and fifth decades. The skin disorder normally starts on the scalp and spreads caudally within a few weeks. It often results in a generalized erythroderma with sharply demarcated islands of sparing ("nappes claires"). A 65-year-old patient with severe PRP showed good clinial improvement after 8 months of treatment when treated with acitretin in combination with phototherapy and systemic gluocorticosteroids.

Pitiriasis rubra pilar clasica del adulto / Classical pityriasis rubra pilaris in the adult

(Se presentan dos casos de pitiriasis rubra pilaris clásica del adulto (Tipo I). Ambos presentaron características clínicas típicas de esta patología. Es nuestro interés hacer una revisión del tema, clasificación, de las nuevas formas de manifestación que tiene esta enfermedad en pacientes inmunosuprimidos y de las terapéuticas actualmente disponibles