Paraneoplastic pityriasis rubra pilaris in association with bronchogenic carcinoma

Pityriasis rubra pilaris (PRP) is an uncommon dermatosis occasionally reported in association with an underlying malignancy. We report a 61-year-old woman in whom PRP preceded the diagnosis of bronchogenic carcinoma by approximately 11 months. The clinical picture in this case included atypical cutaneous signs and mucous membrane involvement. Curative treatment of the malignancy with radiotherapy led to resolution of the mucocutaneous changes within months. A presentation of PRP, particularly if atypical, or in an older patient, should prompt consideration of an associated internal malignancy.     

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

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.     

Photoaggravated pityriasis rubra pilaris

Pityriasis rubra pilaris (PRP) is a rare papulosquamous condition with an estimated incidence of one in 35,000 to one in 50,000. Psoralen and ultraviolet A (UVA) therapy has been used in its treatment but some patients are reported to be clinically photosensitive. We describe the photoinvestigation of a patient with PRP in whom sensitivity to broadband UVA was demonstrated.     

Photosensitive pityriasis rubra pilaris

Exacerbation of pityriasis rubra pilaris (PRP) with ultraviolet exposure is well recognized. However, the initial presentation of PRP in a photoexposed distribution is infrequently reported, and only rarely confirmed with phototesting. We describe such a case of photosensitive PRP. Phototesting revealed exquisite ultraviolet (UV) sensitivity, which was particularly marked to UVB, a feature shared with previously reported cases.     

司库奇尤单抗成功治疗毛发红糠疹一例并文献复习

毛发红糠疹常规治疗包括系统应用维A酸类药物、甲氨蝶呤、环孢素,外用糖皮质激素以及物理治疗等,但效果并不理想.近年来,国外有应用司库奇尤单抗治疗毛发红糠疹的病例报道,且取得了较好的疗效,但国内未见相关报道.本文报道司库奇尤单抗成功治疗毛发红糠疹一例并对相关文献进行复习.     

误诊的幼年型毛发红糠疹一例

患儿男,8岁.因全身多处皮疹伴瘙痒2月余就诊.患儿2个多月前无明显诱因双手背部出现少许红色扁平丘疹、斑块,伴轻度瘙痒,未予重视.后类似皮疹很快播散增多至头面部、双手掌、前臂、肘、膝及臀部,对称分布并融合成片,有少许鳞屑,说话、握拳等活动时有明显皮肤紧绷感.患儿反复在当地诊所及医院就诊,考虑湿疹?银屑病?扁平苔藓?予外用糖皮质激素药膏治疗,皮疹改善不明显,遂来我院就诊.既往体健,其父母非近亲结婚,家族中无类似疾病.     

Circumscribed juvenile pityriasis rubra pilaris

We report the case of a 20-year-old woman with a 10-year history of circumscribed juvenile-onset pityriasis rubra pilaris (PRP, type IV). Our patient had well-defined keratotic follicular papules on an erythematous base located on the extensor aspects of the extremities and dorsal aspects of the feet but no involvement of the palms and soles. Although most cases of type IV PRP follow a favourable course with spontaneous resolution of the lesions, this case demonstrates that circumscribed juvenile PRP can be more persistent and lasts several years.     

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.     

Systemic therapies of pityriasis rubra pilaris: a systematic review

Pityriasis rubra pilaris (PRP) is a rare papulosquamous disorder. Treatment is challenging; the armamentarium consists of topical corticosteroids, phototherapy, classic systemic treatments such as retinoids or immunosuppressive drugs, and most recently biologicals. However, the relative effectiveness of therapies is unclear. Our objective was to review the published literature on systemic treatment of PRP. A systematic review was conducted on PubMed and the Cochrane Library up to 5 September 2017. Studies evaluating any systemic treatments of PRP (except for historical treatments) were included. Overall, 182 studies met the predefined inclusion criteria, and reported on 475 patients and 652 courses of treatment. 42.0 % (225/514) of all patients treated with retinoids achieved an excellent response (isotretinoin: 61.1 % [102/167], etretinate: 47 % [54/115], and acitretin: 24.7 % [43/174]) compared to an excellent response rate of 33.1 % (53/160) with methotrexate. Therapy with biologicals was successful in 51.0 % of patients (71/133) (ustekinumab: 62.5 % [10/16], infliximab: 57.1 % [28/49], etanercept: 53.3 % [16/30], and adalimumab: 46.4 % [13/28]). This review balances effectiveness, side effects, experience, and drug costs in order to suggest a treatment regimen starting with isotretinoin as first‐line, methotrexate as second‐line and biologicals as third‐line treatment for this difficult‐to‐treat dermatosis.