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.

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.

Pitiriasis rubra pilaris en niños:informe de 2 casos / Pityriasis Rubra Pilaris in children: 2 case reports

RESUMEN Antecedentes: La pitiriasis rubra pilaris es una enfermedad infrecuente pápulo-escamosa crónica en la que existe un trastorno de la queratinización de la epidermis, caracterizada por pápulas foliculares hiperqueratósicas con tendencia a formar placas de coloración asalmonada, descamativas, con islas de piel sana asociadas a queratodermia palmo plantar. Presenta una distribución bimodal en la primera y sexta década de la vida, afectando a ambos sexos por igual. Su etiopatogenia es desconocida, se ha postulado una respuesta inmune anormal ante diferentes estímulos antigénicos, así como alteración del metabolismo de la vitamina A. Se ha clasificado en 6 tipos en base a su presentación, edad de inicio, curso y pronóstico, tratándose en forma tópica o sistémica. Casos clínicos: Se presentan dos casos en pacientes de 10 y 2 años de edad, con manifestaciones clínicas correspondientes al tipo juvenil circunscrito, que es el más frecuente en edad pediátrica y juvenil clásico respectivamente, con histopatología compatible y excelente respuesta al tratamiento tópico. Conclusiones: Aunque la pitiriasis rubra pilaris es una patología rara, deberá ser sospechada si el cuadro clínico es sugestivo, y si es compatible su histopatología. Debe considerarse que el tratamientotópico puede ser suficiente para lograr la resolución del cuadro.

ABSTRACT Background: Pityriasis rubra pilaris is an infrequent chronic papulosquamous disease in which there is a disorder of keratinization of the epidermis, characterized by hyperkeratotic follicular papules with a tendency to form salmon-colored, scaly plaques, with islands of healthy skin associated with palmoplantar keratoderma. It presents a bimodal distribution in the first and sixth decades of life, affecting both sexes equally. Its etiopathogenesis is unknown, an abnormal immune response has been postulated to different antigenic stimuli, as well as alteration of the metabolism of vitamin A. It has been classified into 6 types based on its presentation, age of onset, course and prognosis, being treated appropriately topical or systemic. Clinical cases: Two cases of 10 and 2 years of age are presented, with clinical manifestations corresponding to the circumscribed juvenile type, which is the most frequent in pediatric and classic juvenile age respectively, with compatible histopathology and excellent response to topical treatment. Conclusions: Although pityriasis rubra pilaris is a rare pathology, it should be suspected if the clinical picture is suggestive and its histopathology is compatible. It should be considered that topical treatment may be sufficient to achieve resolution of the condition.

Acantholytic pityriasis rubra pilaris associated with topical use of imiquimod 5%: case report and literature review

Abstract Topical use of immune response modifiers, such as imiquimod, has increased in dermatology. Although its topical use is well tolerated, it may be associated with exacerbations of generalized cutaneous inflammatory diseases, possibly through the systemic circulation of pro-inflammatory cytokines. This report describes a case of development of pityriasis rubra pilaris, a rare erythematous-papulosquamous dermatosis, in a woman aged 60 years during treatment with imiquimod 5% cream for actinic keratosis. It evolved with erythrodermic conditions and palmoplantar keratoderma, presenting progressive clinical resolution after the introduction of methotrexate. The authors emphasize the importance of recognizing possible systemic reactions associated with the topical use of imiquimod.

Familial Pityriasis Rubra Pilaris in Siblings / 대한피부과학회지

No abstract available.

Recalcitrant Pityriasis Rubra Pilaris Treated with Ustekinumab / 대한피부과학회지

No abstract available.

Pitiriasis rubra pilaris atípica en pediatría. A propósito de un caso / Atypical pityriasis rubra pilaris in pediatrics. A case report

Resumen Pitiriasis rubra pilaris es una dermatosis eritematoescamosa infrecuente, de etiología desconocida producida por una alteración en la queratinización de la epidermis. Presenta una distribución bimodal con mayor incidencia en la primera y sexta década de vida. Posee una clínica heterogénea clasificada en 6 subtipos según Griffiths, de acuerdo a su presentación clínica y pronóstico. Sus principales hallazgos son pápulas hiperqueratósicas foliculares, queratodermia palmoplantar y placas eritematoesmamosas rojo-anaranjadas que pueden progresar a eritrodermia, con islas de piel sana. En niños las manifestaciones clínicas más frecuentes son la III y IV de la clasificación de Griffiths, según distintos estudios. La histología no es específica pero apoya el diagnóstico. Existen múltiples opciones terapéuticas según la extensión y severidad del cuadro. Presentamos el caso de un preescolar de 5 años de edad con diagnóstico de PRP atípica asociado a eritema extenso, con buena respuesta a corticoides sistémicos y posteriormente a retinoides tópicos.

Summary Pityriasis rubra pilaris (PRP) is an unusual erythematous squamous dermatosis of unknown etiology, caused by an alteration of keratinization in the epidermis. This disease presents a bimodal distribution, being its incidence greater in the first and sixth decade of life. It has a heterogeneous clinical manifestation, and, according to Griffiths, has been classified into 6 subtypes, based on clinical features and prognosis. The typical manifestations of this disease are follicular hyperkeratotic papules, palmoplantar keratoderma and orange-red scaly plaques that can progress to erythroderma, with islands of sparing. According to different studies, the most frequent clinical manifestations in children are type III and IV according to Griffiths classification. Histology is not specific but supports diagnosis. There are multiple therapeutic options, depending on the extension and severity of the disorder. This review presents the case of a 5-year- old child case with a diagnosis of atypical PRP associated with extensive erythema, his response to treatment of systemic corticosteroids and later to topical retinoids being good.

Tinea Versicolor Mimicking Pityriasis Rubra Pilaris caused by Malassezia globosa / 대한의진균학회지

Tinea versicolor (TV) is a common fungal skin disease caused by the Malassezia species. This disease usually presents as hypopigmented- or hyperpigmented coalescing scaly macules, papules, patches or plaques on the trunk and upper arms. Herein, we report a rare clinical manifestation of TV in a 29-year-old man presenting with marked follicular, erythematous, and hyperkeratotic papules on the trunk with erythematous scaly macules and patches on the upper extremities with intermittently spared skin. We initially suspected pityriasis rubra pilaris, however, skin biopsy results and mycological examination revealed TV. Polymerase chain reaction-based sequence analysis revealed Malassezia globosa. The patient was successfully treated with oral itraconazole and topical terbinafine.

A Case of Juvenile Pityriasis Rubra Pilaris Clinically Improved with Cyclosporine / 대한피부과학회지

No abstract available.

Pityriasis Rubra Pilar and hypothyroidism

Pityriasis Rubra Pilaris (PRP) is a chronic and rare papulosquamous disorder. Treatment of Pityriasis Rubra Pilaris is based on empiric evidence because of several doubts regarding its etiology and also because of its relative rarity, making randomized studies difficult to perform. Some factors suggest that the metabolism of vitamin A is involved in pathogenesis. We report a case of Pityriasis Rubra Pilaris associated with autoimmune hypothyroidism which presented rapid and complete response after thyroid hormone replacement, without any association with other systemic treatment. In literature there are only three other reports of significant improvement of the lesions after hormonal correction. Deficiency of thyroid hormone inhibits the conversion of carotene into vitamin A, which would be responsible for the occurrence of Pityriasis Rubra Pilaris in this patient.

Pityriasis Rubra Pilaris Treated with Infliximab / 대한피부과학회지

Pityriasis rubra pilaris (PRP) is a chronic papulosquamous disorder of unknown etiology, which may pose therapeutic challenges. There is currently no universally effective treatment for PRP, and some cases are resistant to multiple topical and systemic therapies. Systemic retinoids, methotrexate, several immunosuppressive agents and phototherapy have all been used with varying degrees of success. Recently, a few reports have appeared in the literature, concerning the use of biologics in combination therapies and/or in refractory PRP cases. We report a case of PRP similar to type II with juvenile onset, which was recalcitrant to traditional topical and systemic therapy. He was successfully treated with anti-TNF-alpha monoclonal antibody, infliximab. The patient showed resolution with minimal disease activity, and was maintained on acitretin and emollients. The response to infliximab in our patient and in the previously reported cases confirms a role of anti-TNF-alpha therapy as an effective option in the treatment of PRP.

A Clinical and Histopathologic Study of Pityriasis Rubra Pilaris / 대한피부과학회지

BACKGROUND: Pityriasis rubra pilaris (PRP)is a rare papulosquamous disorder of unknown etiology. Although the clinical and histopathologic features of PRP are relatively well known in western patients, there are few available studies in Korea. OBJECTIVE: The purpose of this study was to evaluate the clinical and histopathologic features of pityriasis rubra pilaris(PRP) in Korea METHODS: The clinical charts, photographies and skin biopsy slides of patients clinically diagnosed as PRP at the initial visit were reviewed. Finally 30 patients were diagnosed as PRP. They were classified into 5 groups according to the Griffiths classification. And then the evaluation of age at onset, sex distribution, clinical feature of the skin lesion, associated symptoms, clinical course and histopathologic features were conducted. RESULTS: 1) The male to female ratio was 1:1.14. The age of onset was varied from 1 to 65 years with two peaks in first and fifth decades. The average ages of onset in adult type and juvenile type were 40.1 and 5.1 years respectively. 2) According to the Griffiths classification, 14(47%) of the patients had type I, 4(13%) had type III, 7(23%) had type IV, and 3(10%) had type V. However, 2(7%)patients could not be classified under any of these five types due to atypical clinical feature or insufficient follow-up periods. 3) The common clinical features during the disease course were follicular hyperkeratosis(83%), knee and/or elbow involvement(83%), palm and/or sole hyperkeratosis(80%). Erythroderma was present in 5 patients(17%) during the disease course. 4) There were no specific symptoms in most cases, but 9 patients had ben suffered from mild itching. 5) 3 patients had a positive family history, and were thought to have inherited autosomal dominant trait. 6) The frequent histopathologic findings were alternating parakeratosis and orthokeratosis (100%), superficial perivascular infiltration(100%), broad rete ridge(97%), acanthosis(93%), hypergranulosis (87%), follicular plugging(73%). CONCLUSION: We investigated the clinical and histopathological features of PRP in Korea. In our study, the clinical and histopathological features of PRP were not different from those observed in western patients.

A Case of Pityriasis Rubra Pilaris in an Infant / 대한피부과학회지

Pityriasis rubra pilaris is a rare papulosquamous and keratotic disease. The orange hue to the erythema, the thickening of the palms and soles and so called 'islands of normal skin' contribute to establish the diagnosis based on morphologic features. We present a 6-month-old male patient with multiple scaly erythematous patches and 'island of normal skin' on the face. There is no palmoplantar keratoderma, nor onychopathy. Histopathologic findings revealed alternating parakeratosis and hypergranulosis in the epidermis and mild perivascular lymphocytic infiltration in the dermis. Unlike to Griffiths classification, he showed good responses to topical steroid and UV phototherapy.

Pityriasis Rubra Pilaris in Down Syndrome

A 23-year-old woman with Down syndrome presented erythematous keratotic plaques with whitish scales on the elbows, forearm, knee and leg. Histopathological findings revealed acanthosis with broad and short rete ridges, alternating orthokeratosis and parakeratosis oriented in both vertical and horizontal directions, and dermal superficial perivascular lymphocytic infiltration, compatible with pityriasis rubra pilaris.

A Clinical Study of 30 Cases of Pityriasis Rubra Pilaris / 대한피부과학회지

BACKGROUND: Pityriasis rubra pilaris (PRP) is a rare disease characterized by widespread follicular acuminate papules. Traditionally, two forms have been described : hereditary (juvenile) and acquired (generally aduIrt). A.substantial contribution, however, suggests that further subdivision is appropriate. OBJECTIVE: The purpose of this study was to investigate clinicopathologic features of PRP and to compare the results of clinical classification with Griffiths' classification. METHODS: The retrospective clinicopathologic study carried out in 30 cases of PRP over the last 30 years. RESULTS: 1. The incidence of type lV was 38.5%. For both type I and III it was 30.8%. The onset ages of the type I, III, and lV were 36.1, 6.0, and 96 years respectively. 2. The most common site of initial involvement was the palms, and the most common involvement site during the diease course was the dorsa of palms and feet. The incidence involved in the whole body was 46.6%. 3. The incidences of insidious and acute onset were 60% and 40% respectively. No case had the family history of PRP. 4. In the histopathologic study, diffuse hyperkeratosis, acanthosis, and mild dermal infiltrations were found in all cases. Follicular pluggings were observed in 93.3% of cases CONCLUSION: We investigated the clinical classifications and clinicopathologic features of PRP. We think that more studies such as prognosis and treatment of PRP should be achieved in the near future.

A Case of Pityriasis Rubra Pilaris Associated with Incidental Acantholysis

Pityriasis Rubra Pilaris is a rare, chronic, mildly inflammatory disease characterized by fine acuminate follicular papules with orange-red to salmon-colored scaling of the skin and erythroderma that surround islands of normal, uninvolved skin, particular in the trunk. Most patients also develop palmoplantar hyperkeratosis in the early course of the disease. Focal acantholysis occurring in pityriasis rubra pilaris is an unusual and incidental histologic finding within the spectrum of histologic change of the disease. No case featuring this concomitant histologic finding has been reported in the Korean literatures up to date. We report a case of focal acantholysis occurring in a patient with the clinical features of pityriasis rubra pilaris which may be considered as an incidental finding.

A Case of Pityriasis Rubra Pilaris Treated with Etretinate / 대한피부과학회지

We report herein a case of pityriasis rubra pilaris in a 39-year-old male, who had erythematous scaly patches on the face, trunk and extremities for one year. The skin lesion showed well defined plaques on the elbow and knee joint areas, and exfoliative hyperkeratotic sheets with fissures on the palms and soles. He had no family and past history of such skin lesion. He was treated with etretinate lmg/kg/day for 1 week initially, and then tapered off 0.5mg/kg/day for 5 weeks with marked improvement. There was no exacerbation and recurrence of the skin lesion for about 1 year.