Juvenile Pityriasis Rubra Pilaris Research Articles

Severe atypical juvenile pityriasis rubra pilaris diagnosed in adulthood with a dramatic improvement with ustekinumab

AbstractPityriasis rubra pilaris (PRP) is a rare skin disease which manifests as a psoriasiform dermatosis and palmoplantar keratoderma and has significant clinical‐histopathological overlap with psoriasis. Recently, several case reports have demonstrated successful treatment of PRP with anti‐IL7A and anti‐IL12/anti‐IL23 monoclonal antibodies. We report a case of atypical juvenile PRP definitively diagnosed during adulthood with presence of CARD14 mutation. This case demonstrates a dramatic improvement with ustekinumab and highlights the role of genetic testing in chronic disease of diagnostic uncertainty.

Sclerodermiform alterations in the hands of an adolescent

Objective Illustrate an typical presentation of a rare dermatosis. Methods Pityriasis rubra pilaris (PRP) is a rare inflammatory papulo-squamous dermatosis. Genetic factors, changes in vitamin A metabolism and infections have been proposed as triggering mechanism of the disease, but its etiology and pathogenesis are still unknown. It has been described in association with HIV, drugs, autoimmune disease and visceral neoplasms. It affects all races and genders equally, with a peak distribution in the first and fifth decades of life. Most case occur sporadically, but familial cases have been reported. Results Adolescent, 18 years old, with report of generalizes pruritic skin lesions since the age of 3 months, treatd only with antihistamines. He denied comorbidities, allergies or family history of skin diseases. On examination, follicular hyperkeratosis alternating with islands of healthy skin on the anterior and posterior trunk, ichthyosiform desquamation on the lower limbs, palmoplantar keratoderma and sclerodermiform alterations on the hands and feet were noted. A biposy of the lesion on the back was performed, which showed psoriasiform dermatitis with orhokeratosis and parakeratosis alternating horizontally. Considering the clinical-pathological correlation, the diagnosis was juvenile atypical PRP subtype. Conclusion The juvenile atypical subtype of PRP is the least frequent (5% of cases) and accounts for most familial cases. It appears in the first years of life and has a chronic course. It is characterized by follicular hyperkeratosis, erythema, ichthyosiform desquamation on the lower limbs, gross palmoplantar keratoderma and sclerodermiform alterations of the hands and feet.

Treatment Options for Juvenile Pityriasis Rubra Pilaris.

Pityriasis rubra pilaris represents a group of familial and acquired disorders of cornification that affect both adult and pediatric patients. Treatment options are difficult to assess through clinical trials, given the rarity of the disorder and its tendency for spontaneous remission. Case reports and case series are therefore the primary means of assessment. Because of the heterogeneity of the disease, there is no universal approach to treatment, and multiple agents may need to be trialed to achieve disease control. At present, topicals are used for most pediatric patients, though monotherapy with topicals is only effective for less severe disease. Despite concerns over their side-effect profiles, oral retinoids are generally accepted as a first-line systemic therapy. However, interleukin-17 inhibitors and ustekinumab, an interleukin-12 and interleukin-23 inhibitor, may soon become first-line systemic treatment as well, given their efficacy and relative safety in trials thus far. Ustekinumab, in particular, is emerging as a first-line agent for patients with pityriasis rubra pilaris with CARD14 gene variations. When these therapies fail, second-line and adjunctive therapies to consider include tumor necrosis factor-alpha inhibitors, methotrexate, and phototherapy. However, further investigation is necessary to assess the safety and efficacy of many of these agents in juvenile pityriasis rubra pilaris.

Juvenile Pityriasis Rubra Pilaris in a 4-year-old Child Treated Successfully with Secukinumab.

Juvenile Pityriasis Rubra Pilaris in a 4-year-old Child Treated Successfully with Secukinumab.

Treatment of Circumscribed Juvenile Type of Pityriasis Rubra Pilaris with Calcipotriol: A Case Report

Introduction: Pityriasis rubra pilaris (PRP) is a group of chronic papulosquamous disorders manifesting hyperkeratotic follicular papules and forming salmon-colored plaques with white scales. It is a rare case worldwide even in Indonesia. Case Presentation: We report a case of circumscribed juvenile PRP in a four-year-old boy successfully treated with calcipotriol ointment. Conclusions: We treated the patient with 0.005% calcipotriol ointment every 12 h and an oral antihistamine. After 10 weeks, lesions were clinically improved.

Cirmcumscribed juvenile pityriasis rubra pilaris in a 5-year-old-girl treated with topical keratolytic and steroid

Cirmcumscribed juvenile pityriasis rubra pilaris in a 5-year-old-girl treated with topical keratolytic and steroid

Refractory pityriasis rubra pilaris treated with etanercept, adalimumab, or ustekinumab: A retrospective investigation.

Pityriasis rubra pilaris (PRP) is a rare, difficult to treat papulosquamous disorder that responds variably to retinoids and immunosuppression. Successful use of biologics for treating PRP has been described in the literature by case reports and a limited number of case series. To provide additional data, we retrospectively analyzed cases of PRP treated with biologics at our institution. We identified seven patients with a clear diagnosis of PRP treated with adalimumab, etanercept, and/or ustekinumab at our institution from January 1, 2014 to April 1, 2017. Six of seven patients had type I, adult acquired PRP, and one had type V atypical juvenile PRP. In response to tumor necrosis factor (TNF)-α inhibition, two patients had marked responses (>75% improvement in involved body surface area), while three patients failed to show any improvement on a TNF-α inhibitor. In two cases of PRP refractory to TNF-α inhibition, ustekinumab resulted in a partial response (<75% improvement) in one patient and no response in the other. Compared to other published data, our cohort was substantially more resistant to treatment with biologics, a finding which may provide valuable perspective for dermatologists managing refractory PRP in the future.

Classic Juvenile Pityriasis Rubra Pilaris Treated with Oral Alitretinoin

Dear Editor: Pityriasis rubra pilaris (PRP) is a papulosquamous disorder of unknown etiology. Although treatment of PRP is challenging, oral retinoids, including acitretin and isotretinoin, are used as first-line treatment. Recently, alitretinoin, a novel panagonist retinoid, has been reported to be effective for PRP. Here, we described a case of refractory PRP that showed remarkable response to alitretinoin. A 37-year-old man, presenting with relapsing skin lesions over the whole body since 1 year of age, had been diagnosed with and treated for PRP since he was 10 years old. He had a familial PRP history on his mother's side. Physical examination at the first visit revealed erythematous pruritic scaly papuloplaques on the trunk and lower extremities, which was not distinctive due to previous treatments in other hospitals (Fig. 1A, B). Palmoplantar keratoderma was mildly observed. Neither follicular hyperkeratosis nor generalized erythroderma with island of normal skin was seen. The skin biopsy from the left thigh revealed regular acanthosis with broad rete ridge, parakeratosis, and perivascular lymphocytic infiltration with occasional eosinophils (Fig. 1C). There was no confluent hypergranulosis or alternating orthokeratosis and parakeratosis. Classic juvenile PRP was diagnosed. Several treatment options were administered for 1 year in our hospital, which included neotigasone 20~30 mg for 13 months, narrow-band ultraviolet-B phototherapy for 6 months, methotrexate 12.5 mg per week for 1 month, prednisolone 0.5~5 mg for 12 months, and superpotent topical steroid for 6 months. However, none of these treatments led to stable remission. Therefore, oral alitretinoin (30 mg daily) was initiated and continued for 5 months, combined with superpotent topical steroid. The skin lesions and pruritus significantly disappeared in 4 weeks and were not aggravated for 5 months (Fig. 2). Also, there were no side effects except mild dryness. Laboratory test did not show any abnormalities including serum cholesterol. The patient stated that alitretinoin was most effective and tolerable compared with other therapies. Therefore, we decided to continue the same dosage of alitretinoin as far as it was well-tolerated. Fig. 1 Clinical findings of pityriasis rubra pilaris presented at the first visit to our hospital. Multiple erythematous scaly papuloplaques on (A) the trunk and (B) the lower extremities. (C) Histopathologic findings revealed regular acanthosis with broad rete ... Fig. 2 No skin lesion was seen on the (A) trunk and (B) lower extremities after the 5 months course of oral alitretinoin. PRP can be clinically divided into 6 subtypes1. It comprises an adult-onset (classic or atypical adult type), juvenile-onset (classic, circumscribed or atypical juvenile type) and human immunodeficiency virus-associated form. Classic juvenile PRP begins in the first 2 years after birth, showing similar features of classic adult type. Systemic retinoids such as acitretin and isotretinoin have antiproliferative, immuno-modulatory, and anti-inflammatory effects that are mediated by intranuclear retinoid acid receptors (RARs). However, alitretinoin (9-cis-retinoic acid) uniquely binds not only to RARs, but also to retinoid X receptors with high affinity. Owing to this dual interaction, alitretinoin may be more effective than conventional retinoids2. Moreover, alitretinoin inhibits production of nitric oxide and proinflammatory cytokines such as tumor necrosis factor-α, interleukin-1β and interleukin-12p403. In addition, alitretinoin results in fewer mucocutaneous side effects than other retinoids2. To our knowledge, only few cases of adult-onset PRP treated with alitretinoin have been reported2,3,4,5. This is the first case of refractory classical juvenile PRP that responded well to alitretinoin. In conclusion, alitretinoin could be a promising alternative for treating refractory classic juvenile PRP.

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.

A Novel Case of Recalcitrant Juvenile Pityriasis Rubra Pilaris with Response to Bexarotene

Pityriasis rubra pilaris (PRP) is generally treated with single or combination regimens of topical agents, systemic retinoids, methotrexate, biologics, various other immunosuppressant agents, and light therapy; however, cases of PRP that are not amenable to these therapies can be challenging to control. We present the case of a male with biopsy-proven juvenile PRP followed for ten years, with failure to respond to all traditional treatment regimens. Significant improvement in the patient’s cutaneous and rheumatological symptoms occurred when the patient was treated with bexarotene (Targretin®). To our knowledge, this represents the first reported successful treatment of PRP with bexarotene. Thus, treatment with bexarotene may represent a beneficial option for cases of PRP recalcitrant to traditional treatment modalities.

Juvenile pityriasis rubra pilaris: a case report with immunohistochemical and electromicroscopic studies

Juvenile pityriasis rubra pilaris: a case report with immunohistochemical and electromicroscopic studies

パルミチン酸レチノール内服が著効した小児毛孔性紅色粃糠疹の１例

1歳，男児。感冒様症状出現の約2週間後より顔面，両側の手，膝蓋，足に粃糠様，小葉状の鱗屑を付する角化性紅斑局面が出現。特徴的な臨床所見と病理組織学的所見より毛孔性紅色粃糠疹と診断。エトレチナート内服，カルシポトリオール軟膏外用を開始したが変化ないため，パルミチン酸レチノール内服に変更。約1ヵ月で皮疹は著明に改善した。本症は血清ビタミンA値が正常下限であったが，不足はしていなかった。パルミチン酸レチノールが著効したのは，ビタミンAの補正によるのではなく，ビタミンAの薬理作用によるものと考えた。

Juvenile pityriasis rubra pilaris: Report of 28 cases in Taiwan

Pityriasis rubra pilaris (PRP) is a papulosquamous dermatosis uncommon in juveniles. Large-scale studies are limited, especially from Asian countries. We sought to analyze the clinical manifestations of juvenile PRP in Taiwanese patients and compare them with reported series in the literature. 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. 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. This study was a retrospective review. Patient compliance with treatment was frequently poor. 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.

Type III juvenile pityriasis rubra pilaris: a successful treatment with infliximab

Type III juvenile pityriasis rubra pilaris: a successful treatment with infliximab

Recurrence of classical juvenile pityriasis rubra pilaris in adulthood: report of a case

Recurrence of classical juvenile pityriasis rubra pilaris in adulthood: report of a case

Surface image analysis of nail alterations in juvenile pityriasis rubra pilaris.

Juvenile pityriasis rubra pilaris (PRP) is a rare disease that may alter the nail aspect. Image analysis after nail shadowing was used to quantify trachyonychia associated with juvenile PRR Methods: The mean roughness (Ra) and the mean depth of roughness (Rz) of fingernails were measured three times at 3-month intervals in nine children suffering from PRR The same measures were taken in 25 age-matched normal individuals. Both profilometric parameters had higher values in the PRP group than in healthy subjects. The abnormal Ra and Rz values in patients showed variations unrelated to chronobiological cycles. Nail shadowing image analysis is a reliable method to assess inconspicuous to moderate nail surface irregularities. In contrast with some other diseases, the aspect of polymorphic nails in PRP does not seem to be under chronobiological influence.

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.

Etretinate therapy in children with severe keratinization defects.

Keratinization defects can be very severe and disfiguring diseases. The development of retinoids such as etretinate has provided us with an effective symptomatic form of oral therapy for these skin conditions. Based on our own experience, we briefly outline the therapeutic potential of etretinate in various keratinization defects (lamellar ichthyosis, Netherton syndrome, Sjögren-Larsson syndrome, mal de Meleda and juvenile pityriasis rubra pilaris). The toxicology of etretinate is reviewed with special regard to the treatment of children. Bone changes such as premature closure of the growth line or other unacceptable side-effects have so far not been observed. Guidelines for patient selection and for the safe treatment of children are given.