[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.

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.

The Incidence of Acantholysis in Pityriasis Rubra Pilaris-Histopathological Study Using Multiple-Step Sections and Clinicopathologic Correlations.

BACKGROUND: The classical histopathological findings in the epidermis of pityriasis rubra pilaris (PRP) do not include acantholysis; however, acantholysis was described in several case reports and a few series of PRP with variable frequencies. We sought to establish the incidence of acantholysis in biopsies from consecutively referred PRP cases using multiple-step sections and clinicopathologic correlations. METHODS: Twenty-three biopsies from 12 consecutively referred patients with classical (type 1) PRP were studied histopathologically. Each specimen was completely step sectioned. The clinical files of the patients were also reviewed. RESULTS: Small foci of acantholysis were observed in some of the step sections of 5 of 23 (22%) biopsies obtained from 4 patients. Three biopsies showed suprabasal acantholysis, 1 of which also demonstrated mild dyskeratosis and 2 showed midepidermal acantholytic foci as well. The remaining 2 biopsies demonstrated midepidermal and subcorneal acantholysis, respectively. Small erosions were described in the physical examination of 2 of the 4 (50%) patients with acantholysis and in 1 of the 8 (12.5%) patients without acantholysis. LIMITATIONS: The number of cases. CONCLUSIONS: Small foci of acantholysis may be found in the minority of PRP biopsies, and it may be related to small erosions clinically in some patients.

An unusual cluster of circumscribed juvenile pityriasis rubra pilaris cases.

Circumscribed juvenile pityriasis rubra pilaris (PRP) is an uncommon dermatosis. We describe the unusual clustering of circumscribed juvenile PRP cases in our pediatric dermatology clinic in 2011. A retrospective chart review was done of patients presenting during the summer of 2011 with classic findings of circumscribed juvenile PRP. Clinical data including past medical and family history, presenting symptoms, infectious disease history and evaluation, biopsy results, and management were recorded. Seven patients, ages 5 to 19 years, all had strikingly similar skin findings of pink to hyperpigmented, well-defined, scaly papules and plaques on their elbows, knees, dorsal hands, ankles, and Achilles tendons. Four of the seven also had palmoplantar involvement. Four were sibling pairs and the other three were unrelated. Streptococcus pyogenes infection was suspected as a trigger in four of the patients. The unusual clustering of this uncommon disease, along with the occurrence in two sibling pairs, suggests that a genetic susceptibility unmasked by an infectious agent may play a role in its pathogenesis.

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.

Pitiriasis rubra pilaris aguda postinfecciosa: una dermatosis mediada por superantígenos / Acute Postinfectious Pityriasis Rubra Pilaris: A Superantigen-Mediated Dermatosis

La pitiriasis rubra pilaris (PRP) aguda post infecciosa es una variante de la forma juvenil de PRP (tipo III de Griffiths) caracterizada por la ausencia de antecedentes familiares, curso agudo relacionado con un episodio febril previo y buen pronóstico. Clínicamente puede simular otras enfermedades mediadas por superantígenos, como los exantemas escarlatiniformes o el síndrome de la escaldadura estafilocócica; sin embargo, su histología y tratamiento son distintos. Presentamos 4 casos de PRP aguda post infecciosa que ilustran las características clínicas de este proceso infrecuente y revisamos los posibles mecanismos fisiopatogénicos subyacentes (AU)

Acute postinfectious pityriasis rubra pilaris (PRP) is a variant of juvenile PRP (Griffiths type III) characterized by no family history, an acute course associated with a prior fever, and good prognosis. Clinical features may resemble other superantigen-mediated diseases, such as scarlatiniform rash or staphylococcal scalded skin syndrome, but its histology and treatment are different. We present 4 cases of acute postinfectious PRP that illustrate the clinical features of this uncommon disease and we review possible underlying pathogenic mechanisms (AU)

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.

Juvenile pityriasis rubra pilaris: report of 28 cases in Taiwan.

BACKGROUND: Pityriasis rubra pilaris (PRP) is a papulosquamous dermatosis uncommon in juveniles. Large-scale studies are limited, especially from Asian countries. OBJECTIVE: We sought to analyze the clinical manifestations of juvenile PRP in Taiwanese patients and compare them with reported series in the literature. METHODS: The diagnosis of juvenile PRP was made based on clinical-histopathologic correlation. The therapeutic response and disease course were followed up by re-examination of the patients or by telephone. RESULTS: A total of 47 patients were identified, with histopathologic confirmation of the clinical diagnosis of juvenile PRP in 28 cases. A preponderance of Griffiths' type IV PRP (85.7%) rather than type III PRP (14.3%) was found. Palmoplantar hyperkeratosis appeared to be a cardinal feature. In patients with type IV PRP, skin lesions in areas other than the elbows/knees and palms/soles were common. Treatment with systemic acitretin in 6 patients failed to effect a dose- or time-dependent improvement. In contrast with other studies, two thirds of our patients with type III and IV juvenile PRP had a protracted course lasting more than 3 years. LIMITATIONS: This study was a retrospective review. Patient compliance with treatment was frequently poor. CONCLUSIONS: Type IV juvenile PRP predominated but our cases showed a wider distribution of skin lesions than is typically described. When children present with an acute onset of diffuse palmoplantar hyperkeratosis, a diagnosis of juvenile PRP should be considered. Because of the divergent clinical manifestations of juvenile PRP in different populations, there is a need to modify and re-evaluate classification systems based on regional differences.

Pityriasis rubra pilaris in childhood: a long-term study of 29 cases.

Pityriasis rubra pilaris (PRP) is a dermatosis of unknown origin with a limited frequency in childhood (0.2% of our hospitalized pediatric patients). During the last 20 years we have observed 31 new cases; follow-up has been conducted in 29 children. The acute self-resolving form seems to be the most frequent in children, compared to adults. No cases of hereditary PRP have been observed, and no relationship between the severity of PRP and its prognosis has been reported. Since juvenile PRP has a relatively rapid course and a spontaneous resolution (a few months), it seems unnecessary to use potentially harmful drugs. Synthetic retinoids may be employed in patients whose disease is both persistent and disabling.

Papulosquamous diseases: a review.

Papulosquamous diseases are a heterogeneous group of disorders whose etiology primarily is unknown. The nosology of these disorders is based on a descriptive morphology of clinical lesions characterized by scaly papules and plaques. The major entities in this group include psoriasis, parapsoriasis (including pityriasis lichenoides et varioliformis acuta), lichen planus, lichen nitidus, lichen striatus, pityriasis rosea, pityriasis rubra pilaris, seborrheic dermatitis, and the Gianotti-Crosti syndrome. Many other conditions may become papulosquamous and should be considered in the differential diagnosis.