Recalcitrant psoriasiform dermatosis of the face: Is it related to pityriasis rubra pilaris?

BACKGROUND: There are patients with recalcitrant psoriasiform plaques that do not fit into conventional categories of facial dermatoses. Our study aims to describe the clinicopathological characteristics of several patients with a unique presentation of persistent psoriasiform facial rashes. METHODS: This retrospective cross-sectional study analyzed clinical and histological data of known cases of recalcitrant psoriasiform dermatosis of the face diagnosed at National Skin Centre, Singapore, over 10 years. RESULTS: There were 8 Chinese patients with mean age at onset of 29 years. Majority had pink to pink-orange well-defined plaques with dry scale (n = 6, 75%), distributed mostly on the cheeks (100%) and chin (n = 7, 88%). Hyperkeratosis, parakeratosis, preserved granular layer and psoriasiform hyperplasia were showed in all biopsies. Other common findings included subtle subcorneal acantholysis, "checkerboard" alternating ortho-/parakeratosis, vacuolated keratinocytes and follicular plugging. All patients showed little treatment response. One patient eventually developed features of type II pityriasis rubra pilaris (PRP). Our study was limited by its small sample size and lack of a pre-existing diagnostic code. CONCLUSIONS: This recalcitrant psoriasiform facial dermatosis seems to be a distinct entity, with consistent and reproducible clinical features and a PRP-like histology, bearing some resemblance to the recently described condition-facial discoid dermatosis.

Pityriasis Rubra Pilaris Type V as an Autoinflammatory Disease by CARD14 Mutations.

Importance: We found CARD14 mutations (2 de novo novel mutations and another previously reported mutation) in 3 of 3 patients with pityriasis rubra pilaris (PRP) type V, but not in patients with PRP of other types. Our findings, combined with the published literature, suggest that type V PRP, both familial and sporadic, can be caused by CARD14 mutations. Detailed clinical observation revealed that all 3 patients displayed unique patchy macular brown hyperpigmentation. Objective: To further determine how often patients with PRP have pathogenic mutations in CARD14 and to elucidate which clinical subtype of PRP is caused by CARD14 mutations. Design, Setting, and Participants: We sequenced the entire coding regions of CARD14 in genomic DNA from patients with 5 clinical subtypes of PRP. The detailed clinical features were analyzed in all the patients. The pathogenicity of each mutation was evaluated by several computational predictions. PRP was classified into 6 subgroups, types I to VI, based on clinical criteria. We categorized all the patients with PRP into the clinical subtypes using the classic PRP classification; 22 cases of PRP with varying subtypes were studied. Main Outcomes and Measures: The prevalence of CARD14 mutations in each subtype of PRP was evaluated. Clinical features and characteristics of patients with PRP with CARD14 mutations were analyzed. Results: Overall 22 patients with PRP were included in our study (12 men, 10 women; mean [SD] age, 26 [18] years). Among 3 patients with PRP type V, all were found to have CARD14 mutations: 2 de novo novel mutations (p.Cys127Ser and p.Gln136Leu), and another previously reported mutation (p.Gly117Ser). All were close to the reported pathogenic domains. In silico analysis of all 3 mutations suggested that they are functionally relevant to pathogenesis. All 3 patients displayed unique patchy macular brown hyperpigmentation additionally to other typical features of PRP. Patients with PRP type I and type IV, 1 patient each, had the rare variants in CARD14. Conclusions and Relevance: Pityriasis rubra pilaris type V is a distinct variant of PRP that is caused by CARD14 mutations. In addition, a rare variant of CARD14 might also be implicated in the pathophysiology of other forms of PRP.

Pityriasis rubra pilaris: clinicopathological study of 32 cases from Lebanon.

BACKGROUND: Pityriasis rubra pilaris (PRP) is an uncommon papulosquamous disorder of unknown etiology. Studies on this condition from our region are lacking. METHODS: To describe the clinical and histopathological findings as well as response to treatment of all patients diagnosed with PRP at the American University of Beirut Medical Center between 1995 and 2010 and compare our findings with those published in the literature. RESULTS: Pityriasis rubra pilaris was diagnosed in 32 patients (16 males, 16 females). Age of onset ranged between 2.5 and 70 years. The majority of patients (n=15) were classified as type I (47%) followed by type III (n=9, 28%) and type IV (n=6, 19%). Based on treatment responses, retinoids appear to be very effective in our population as all patients treated with isotretinoin or acitretin had excellent response. In addition to checkerboard alternating orthokeratosis/parakeratosis, which was observed in 31 cases, interesting features, including the presence of follicular plugging in all 21 cases in which follicles were available for examination, eosinophils in 12 cases, and focal acantholysis in three cases were observed. CONCLUSIONS: Features of patients with PRP in our study are generally comparable to those published in the literature, with minor differences. Microscopically follicular plugging, in addition to checkerboard alternating orthokeratosis/parakeratosis, may serve as clues to PRP diagnosis. The presence of eosinophils and focal acantholysis, observed in a few cases, should not exclude PRP diagnosis.

Pitiriasis «rubra pilaris» localizada / Pityriasis rubra pilaris localized

Incluimos la pitiriasis rubra pilaris (PRP) dentro del grupo de las dermatosis papuloescamosas hiperqueratósicas de origen desconocido. Aunque poco frecuente, se han constatado dos picos de incidencia en la primera y en la quinta década de la vida, lo cual determina su clasificación en cinco grandes grupos, los dos primeros típicos en la edad adulta y los tres restantes en la edad juvenil. En los últimos años se ha incluido un sexto grupo, asociado al virus de la inmunodeficiencia humana (VIH), independientemente de la edad. No existe un tratamiento estandarizado para la PRP, pero por lo general en casos localizados suelen emplearse tratamientos tópicos, entre los que incluimos los corticoides, los derivados de la vitamina D o los retinoides; en casos más extensos podría recurrirse a tratamientos sistémicos, y como primera elección los retinoides orales(AU)

Pityriasis rubra pilaris (PRP) is an uncommon papulosquamous keratotic dermatosis of unknown origin. It has a bimodal distribution of age onset concentrating in the first and fifth decades. Classification of PRP includes 5 types: types I and II represent the forms of adult PRP, respectively, whereas types III, IV, and V are seen in juveniles. In recent years, a type VI PRP associated with HIV infection has been proposed. A universal standard treatment for PRP is lacking. Topical therapy is the treatment of choice for patients with mild type III and type IV PRP, including corticoids, vitamin D analogue ortopical retinoids. Systemic therapy is reserved for patients with severe type III and type V. Acitretin and isotretinoin are the treatment of choice(AU)

Pityriasis rubra pilaris: evolution of challenges in promising treatment options.

Pityriasis rubra pilaris is an uncommon inflammatory dermatosis that is well recognized across the globe. Erythroderma is a common presentation. A precise diagnosis of pityriasis rubra pilaris is based on morphologic features and is classified into 6 types: classic adult onset (type I), atypical adult (type II), classic juvenile (type III), circumscribed juvenile (type IV), atypical juvenile (type V), and human immunodeficiency virus-associated (type VI). Several conventional systemic and/or topical treatments are currently in use. Largely, their results are unsatisfactory and limited by long-term toxicity. The authors investigate the efficacy of a wide spectrum of drugs by examining historical (archive) and promising (modern) treatment modalities for the treatment of pityriasis rubra pilaris.

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.

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: 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 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.

Adult onset pityriasis rubra pilaris.

Pityriasis rubra pilaris (PRP) has always been an intriguing topic ever since its inception. It is a group of chronic disorders characterized by reddish orange plaques with pityriasiform scaling showing follicular keratoses, palmoplantar keratoderma, and sometimes, erythroderma. It occurs all over the world but with racial variations. Its incidence might vary and the age at onset, behavior, clinical appearance, and prognosis are considered to be very important for its classification. It may manifest either as Type I classical adult onset PRP, Type II atypical adult (onset) PRP, or Type VI PRP (HIV-associated PRP pityriasis rubra pilaris) in contrast to classical juvenile (Type III) and circumscribed juvenile (Type IV) encountered among children. Its diagnosis is largely clinical with microscopic pathology being a useful supplement, but it continues to be a therapeutic dilemma. We review the epidemiology of adult onset PRP here and take stock of the prevalent treatment options.

Pityriasis rubra pilaris, type 1.

A 57-year-old woman presented with a history of dry skin with an associated sensation of burning and itching. It had been previously diagnosed as psoriasis. Clinical and histopathologic examination were consistent with pityriasis rubra pilaris, and treatment consisted of acitretin and narrow-band ultraviolet B phototherapy. Pityriasis rubra pilaris is a papulosquamous disorder of unknown etiology, which can be treated with retinoids, methotrexate, cyclosporine, and narrow-band phototherapy.

Pityriasis rubra pilaris: a clinical review.

Pityriasis rubra pilaris is a skin condition with many different clinical presentations. History, histology, clinical presentation, its different classified forms, treatments, and differential diagnoses are reviewed.

Pitiriasis rubra pilaris eritrodérmica: buena respuesta al tratamiento con acitretin / Erythrodermic pityriasis rubra pilaris: good response to treatment with acitretin

La Pitiriasis Rubra Pilaris es una dermatosis papuloescamosa crónica, de etiología desconocida, caracterizada por la presencia de pequeñas pápulas foliculares, placas rojo-anaranjadas y queratodermia palmoplantar. Las lesiones suelen ser simétricas, dejando islotes de piel sana, y puede evolucionar a eritrodermia. Se presenta el caso de un paciente de 53 años de edad con pitiriasis rubra pilaris eritrodérmica. Se revisan las características clínicas e histopatológicas de la enfermedad y sus opciones terapéuticas.

A review of pityriasis rubra pilaris and rheumatologic associations.

Pityriasis rubra pilaris (PRP) is a rare group of hyperkeratotic, papulosquamous disease that can be acquired or inherited. There have been reported cases of rheumatologic associations, mainly arthritis and dermatomyositis. In this review article, we will explore the clinical presentation and classification, rheumatologic associations and treatment modalities of PRP. In addition, we will also report a case of PRP with seronegative arthritis.

[Acute juvenile pityriasis rubra pilaris in a 2-year-old child]. / Akute Juvenile Pityriasis Rubra Pilaris bei einem 2jährigen Kind.

Pityriasis rubra pilaris (PRP) is a rare idiopathic erythematosquamous disorder. Griffiths proposed five clinical and prognostic forms: (1) classic adult, (2) atypical adult, (3) classic juvenile, (4) circumscribed juvenile and (5) atypical juvenile. A 2 1/2 year old boy presented with type 3 PRP; as is typical, the skin eruption was preceded by an infectious disorder. Although type 3 PRP normally shows spontaneous resolution after several months to years, our young patient had a severe and protracted course, only responding to systemic retinoids.