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.

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.

Pitiriasis rubra pilaris: a propósito de un caso / Pityriasis rubra pilaris: case report

RESUMEN Se presenta caso de paciente masculino de 9 años de edad, fototipo VI acude a consulta especializada de Dermatología en el Hospital pediátrico William Soler por lesiones eritematoescamosas dispuestas en placas localizadas en ambas rodillas, acompañadas de queratodermia palmo plantar. Se realizan exámenes complementarios y biopsia de piel concluyéndose caso como pitiriasis rubra pilaris circunscrita juvenil. Esta dermatosis es de infrecuente presentación, desafío terapéutico por ausencia de estandarización internacional y cursa con una evolución impredecible.

ABSTRACT We present the case of a 9-year-ol, phototype 3, who attended a specialized dermatology consultation at the William Soler pediatric hospital due to erythematosquamous lesions arranged in plaques located on both knees accompanied by palmoplantar keratoderma. Laboratory tests and skin biopsies were carried out, concluding case as a juvenile and circumscribed pityriasisrubra pilaris. This is an infrequent dermatosis, a therapeutic challenge because of the absence of international standardized treatments and it has an unpredictable evolution.

Pityriasis Rubra Pilaris classic adult: a rare pathology / Pitiriasis rubra pilaris clásica del adulto: una patología poco frecuente

RESUMEN La Pitiriasis Rubra Pilaris es una enfermedad papuloescamosa, secundaria a un trastorno de la cornificaciónde causa desconocida, se manifiesta clínicamente por pápulas hiperqueratósicas y queratodermiapalmoplantar. Existen diferentes formas de clasificar la enfermedad,en función de la edad deinicio, morfología, curso clínico y pronóstico. La forma de presentación más frecuente corresponde a la clásica del adulto (Tipo I), alrededor del 55% de todos los casos. Por tratarse de una enfermedad con baja incidencia, de etiología poco clara y con la posibilidad de remisión espontánea, no existe un tratamiento universalmente aceptado, la mayor parte de la evidencia proviene de reportes de casos, series de casos y algunos estudios retrospectivos. Presentamos el caso clínico de una paciente de 57 años de edad con diagnóstico de Pitiriasis Rubra Pilaris con respuesta favorable a retinoides.

SUMMARY PityriasisRubra Pilaris is a papulosquamous disease, secondary to a cornification disorder of unknown cause, clinically manifested by hyperkeratosic papules and palmoplantar keratoderma. There are different ways to classify the disease, it corresponds to six subtypes depending on the age of onset, morphology, clinical course and prognosis. The most frequent form of presentation corresponds to the classic one of the adult (Type I), around 55% of all cases. Due to it is a low incidence disease, with an unclear etiology and the possibility of spontaneous remission, there is no universally accepted treatment and most of the evidence comes from individual case reports, small case series, and some retrospective studies. We present the clinical case of a 57-year-old patient diagnosed with PityriasisRubra Pilaris with a favorable response to retinoids.

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.

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

No abstract available.

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.

Pitiriasis rubra pilaris juvenil: presentación de un caso / Pityriasis rubra pilaris: a case report

La pitiriasis rubra pilaris es un trastorno de la queratinización infrecuente, que se caracteriza por pápulas hiperqueratósicas y queratodermia palmoplantar. Se presenta con igual frecuencia en ambos sexos y tiene aparición bimodal, en la primera década de la vida y luego en la quinta y sexta. Presentamos el caso de una joven que presentó la enfermedad desde los cinco años, con evolución crónica de la misma y que respondió favorablemente al tratamiento tópico.

Pityriasis rubra pilaris is a rare disorder of keratinization, characterized by hyperkeratotic papules and palmoplantar keratoderma. It occurs equally in both sexes and has a bimodal appearance, in the first decade of life and then in the fifth and sixth decade. The classification of this disease represents a challenge due to the variability of the clinical findings. We present the case of a young woman who presented the disease in early age, had a chronic evolution and responded favorably to topical treatment.

Queratosis pilar simil asociado a uso de nilotinib / Keratosis pilaris associated with nilotinib use

Nilotinib es un inhibidor altamente selectivo de BCR-ABL tirosina kinasa usado para el tratamiento de Leucemia mieloide crónica. Las reacciones cutáneas fueron uno de los efectos adversos no hematológicos más frecuentemente reportados en relación al uso de esta droga. El presente artículo documenta el caso una paciente femenina de 17 años de edad diagnosticada con Leucemia mieloide crónica que había estado en tratamiento con Nilotinib por 5 meses desarrollando una reacción tipo queratosis pilar. La paciente fue tratada con medidas generales, Urea 15% y antihistamínicos, con cese del prurito. Es importante reconocer las reacciones cutáneas asociadas al uso de Nilotinib para así otorgar alivio oportuno de los síntomas con el fin de lograr una mejor adherencia al tratamiento de la Leucemia mieloide crónica y mejorar la calidad de vida del paciente.

Nilotinib is a highly selective inhibitor of BCR-ABL tyrosine kinase. It is used as a treatment for chronic myelogenous leukemia (CML). Cutaneous reactions are one of the most common non-hematologic reported adverse effects. The present article documents the case of a 17-year-old female patient diagnosed with CML. She was treated with nilotinib for 5 months and developed a keratosis pilaris-like reaction. The patient was treated with general measures, topical 15%-urea and antihistamines with improvement and cessation of pruritus. It is imperative to recognize the cutaneous adverse effects associated with the use of new oncologic treatments such as nilotinib.

A Case of Cardiofaciocutaneous Syndrome / 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.

Estudio retrospectivo de pacientes con alopecia fibrosante frontal / Retrospective study of patients with frontal fibrosing alopecia

Introducción: La alopecia fibrosante frontal, considerada por algunos autores como un subtipo de liquen plano pilaris, corresponde a una alopecia cicatricial primaria, que se presenta en mujeres adultas y se caracteriza por el retroceso simétrico de la línea de implantación fronto temporal. Se presenta un estudio descriptivo de pacientes con Alopecía fibrosante frontal atendidos en este centro. Objetivos: Evaluar características clínicas, dermatoscópicas e histopatológicas de pacientes con alopecia fibrosante frontal. Materiales y métodos: Estudio retrospectivo de pacientes con diagnóstico clínico-histopatológico de alopecia fibrosante frontal atendidos en nuestro centro desde Julio 2010 a Octubre 2012. Resultados: Se encontraron 57 casos con diagnóstico histológico de alopecia cicatricial, de los cuales 8 (14 por ciento) se diagnosticaron como Alopecia Fibrosante Frontal. El 100 por ciento correspondieron a mujeres, el promedio de edad fue de 45 años. Sólo la mitad de los casos comenzó con la alopecia luego del climaterio. El hallazgo clínico más frecuentemente encontrado fue el retroceso de la línea de implantación frontotemporal (87,5 por ciento); y en segundo lugar la disminución difusa de densidad capilar (37,5 por ciento) y alopecia de la cola de las cejas (50 por ciento). No se reportó ningún caso asociado a liquen plano cutáneo o de mucosas. Los hallazgos más relevantes a la dermatoscopía fueron: eritema perifolicular (50 por ciento) e hiperqueratosis folicular (25 por ciento). A la histopatología, los principales hallazgos fueron el infiltrado inflamatorio linfocitario perifolicular (50 por ciento) y fibrosis concéntrica perifolicular (100 por ciento). Discusión: La Alopecia fibrosante frontalrepresenta el 14 por ciento de las alopecias cicatriciales primarias en nuestra serie. La edad al diagnóstico fue inferior a lo reportado en la literatura. El principal hallazgo clínico fue el retroceso de la línea de implantación frontotemporal...

Introduction: Frontal fibrosing alopecia, considered by some authors as a subtype of lichen planopilaris, is a scarring alopecia that usually involves adult women and is characterized by the symmetric recession of fronto-temporal hairline. Objectives: Characterize the clinical, dermoscopic and histological features of frontal fibrosing alopecia. Materials and Methods: We conducted a retrospective study of patients with clinical and histological diagnosis of Frontal Fibrosing Alopecia treated in our institution from July 2010 to October2012. Results: 8 out of 57 cases with histological diagnosis of scarring alopecia, had diagnosis of frontal fibrosing alopecia (14 percent). 100 percent were women, with mean age of 45 years. 50 percent of patients began with alopecia in the post-climacteric period. Recession of frontotemporal hair-line was the most common finding (87.5 percent), associated with reduced capillary density (37.5 percent) and loss of eyebrows (50 percent). In our study, none of the patients had other signs of lichen planus. At dermoscopy, most common finding were perifollicular erythema (50 percent) and follicular hyperkeratosis (25 percent). Most common histological findings were a perifollicular lymphocytic inflammatory infiltrate (50 percent) and perifollicular concentric fibrosis (100 percent). Discussion: Frontal fibrosing alopecia represents 14 percent of scarring alopecia in our series. Age at diagnosis was lower than reported in the literature, and only 50 percent of women presented alopecia in the postmenopausal period. The main clinical finding was the recession of frontotemporal hairline...

Ulerythema ophryogenes: a case report / 中华皮肤科杂志

A case of ulerythema ophryogenes (UO) is reported.A 12-year-old boy presented with erythema and follicular papules on the eyebrows and cheeks for 7 years.The lesions started as follicular papules surrounded by erythema,then spread symmetrically to the cheeks and forehead followed by the loss of eyebrows.There was no complaint of pruritus.Physical examination showed pinhead- to grain-sized,smooth,slightly indurated follicular hyperkeratotic papules surrounded by erythematous halo on the eyebrows,forehead and cheeks.Both eyebrows were nearly completely lost.Histological analysis of lesions from eyebrows revealed dilated follicular infundibulum with orthokeratotic plugs,sparse perivascular and perifollicular lymphohistiocytic infiltrate,widened and sclerotic collagen fibers in the dermis.According to the clinical manifestations and histopathological findings,the patient was diagnosed with ulerythema ophryogenes,and given oral vitamin A 2.5 million unit once a day,vitamin E 100 mg once a day,topical vitamine E cream twice a day,0.025％tretinoin ointment once at night.Two weeks later,the lesions improved.

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.

Pitiriasis rubra pilaris eritrodérmica: algo para no olvidar / Erythrodermic pityriasis rubra pilaris

La Pitiriasis Rubra Pilaris (PRP) es una dermatosis papuloescamosa crónica, de etiología desconocida. Se caracteriza por la presencia de pápulas foliculares hiperqueratósicas que coalescen formando placas eritematoescamosas, dejando islotes de piel sana entre las lesiones. Puede llegar a una eritrodermia. Se clasifica en base a la edad de presentación, características morfológicas, evolución y pronóstico. Existen múltiples opciones de tratamiento descritas en la literatura, siendo los retinoides sistémicos el tratamiento de primera línea en estos pacientes. Presentamos dos casos de pacientes con PRP eritrodérmica tratados exitosamente con Acitretín y revisión de la literatura a la fecha.

Pityriasis Rubra Pilaris is a chronic inflammatory dermatosis of unknown etiology, characterized by the presence of multiple follicular papules that coalesce into large erythematous or salmon colored plaques with islands of non-involved skin between them. It can eventually evolve into erythroderma. Descriptions and therapeutic experiences are mainly based on case reports. Today retinoids have become de first line treatment in these patients. We present two cases of erythrodermic PRP treated successfully with Acitretin and an updated review of the literature.