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.     

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.     

毛发红糠疹研究进展

毛发红糠疹是一种少见的红斑鳞屑性皮肤病,其发病可能与遗传、生化代谢和内分泌异常、免疫反应、恶性肿瘤、药物和感染有关。治疗包括口服维A酸、外用糖皮质激素和保湿剂,TNF-α 拮抗剂等,本文就其病因、发病机制、治疗等方面进行综述。     

毛发红糠疹28例临床分析

目的:分析毛发红糠疹的临床特点和疗效。方法:回顾性分析28例毛发红糠疹患者的临床症状和治疗。结果:28例毛发红糠疹皮损表现为毛囊角化性丘疹和掌跖角皮症。给予阿维A 10mg每日2次口服,配合甘草酸二胺150 mg静滴及0.1%维A酸乳膏外用治疗2周后,皮损好转,4周后皮损明显消退。结论:毛发红糠疹临床表现及组织病理学检查有特征性改变。阿维A治疗有效。     

毛发红糠疹的治疗进展

毛发红糠疹病因及发病机制不明,通常对传统治疗抵抗。由于缺乏高质量的证据,目前尚无治疗指南,维A酸类药物被普遍作为一线治疗,近年来生物制剂被证明在严重难治性病例中有快速、显著的疗效。本文就PRP治疗方法进行综述。     

Methotrexate treatment in a case of juvenile pityriasis rubra pilaris

An 8‐year‐old boy who was initially diagnosed with plaque psoriasis failed management with topical therapies and skin biopsy confirmed the suspected diagnosis of juvenile pityriasis rubra pilaris (PRP). Pityriasis rubra pilaris is a rare inflammatory disorder of the skin characterized by follicular keratotic papules coalescing into plaques, along with palmoplantar keratoderma. Treatment modalities include topical and systemic therapies, although previous studies have not shown much benefit with methotrexate in children. We present a case in which methotrexate led to significant improvement of the skin findings in a child with type IV juvenile pityriasis rubra pilaris.     

A case of pityriasis rubra pilaris associated with membranous nephropathy

Pityriasis rubra pilaris (PRP) is a rare idiopathic dermatosis which may be associated with autoimmune diseases, HIV infection, and internal malignancies. Its association with renal diseases is, however, much less recognized. We report a case of PRP with associated membranous nephropathy (MN), which resolved spontaneously with resolution of the dermatosis. This is only the second reported association between PRP and MN of which we are aware. Further reports of such an association will strengthen the evidence for the two conditions being linked and may thereby shed light on the pathogenesis of both PRP and MN.     

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.     

Surface image analysis of nail alterations in juvenile pityriasis rubra pilaris

Background/aims: 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. Results: 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. Conclusions: 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.     

Ustekinumab treatment of pityriasis rubra pilaris: A report of five cases

Pityriasis rubra pilaris (PRP) is a rare, chronic, inflammatory skin disease of unknown etiology. Patients refractory to conventional therapies have been treated successfully with biologic drugs such as anti‐tumor necrosis factor agents. Recently, a role of the interleukin‐23/T‐helper 17 axis in PRP has been described. Our objective was to assess the effectiveness of ustekinumab in five patients with adult‐onset PRP refractory to conventional therapies. In the present study, four patients had type I and one patient type II adult‐onset PRP. They were treated with three s.c. doses of ustekinumab at weeks 0, 4 and 16. Clinical response was evaluated monthly during treatment up to a 15‐month follow‐up period. All patients promptly showed a decrease in erythema, follicular hyperkeratosis and scaling. After three injections, complete remission of skin lesions was achieved in four out of five cases and a significant clinical improvement was shown in one case. To the best of our knowledge, this is the largest case series reported on ustekinumab treatment in PRP. Our results, in addition to previous studies from other groups, suggest that ustekinumab may be a possible first‐line treatment for PRP patients refractory to conventional therapies.     

Treatment of refractory adult‐onset pityriasis rubra pilaris with TNF‐alpha antagonists: a case series

Background Pityriasis rubra pilaris (PRP) is a rare inflammatory dermatosis with frequent clinical presentation as erythroderma. Conventional systemic treatment is often unsatisfactory and limited by long‐term toxicity. The use of tumour necrosis factor (TNF) antagonists has been reported previously in single cases, but lacking long‐term follow‐up or comparison between different biological agents. Objectives To assess the long‐term efficacy and safety of TNF‐alpha antagonist, infliximab and etanercept, either in monotherapy or in combination therapy of severe, refractory adult‐onset PRP. Methods Seven patients of adult‐onset PRP, six newly diagnosed type‐I and 1 type‐II, which were resistant or ineligible to conventional systemic treatment, received a single course of infliximab or etanercept therapy, alone or in combination with low‐dose acitretin (>0.25 mg/kg/daily). After complete remission and treatment discontinuation, a follow‐up period of 12 months was evaluated for relapses. Results Six patients obtained complete remission after a single course of anti‐TNF‐alpha therapy: mean therapy duration was 19.3 weeks (range 6–48 weeks). All patients obtained significant clearing (>75% of body surface area) of skin lesions at week 12. Two patients with marked keratoderma developed localized disease recurrence during treatment. During follow‐up, only a single patient, affected by type II PRP, had disease relapse. Conclusions Both TNF‐alpha antagonists proved successful for the treatment of refractory, adult‐onset PRP, yielding complete and persistent clinical responses in type‐I PRP. Infliximab was associated with a more rapid onset of action, while treatment duration was comparable with etanercept. PRP type II warranted long‐term therapy and showed relapse after drug discontinuation.     

Lichen‐planopilaris‐like scarring pattern in a patient with alopecia and pityriasis rubra pilaris

Pityriasis rubra pilaris (PRP) is an erythematous‐desquamative dermatitis that is sometimes associated with non‐scarring alopecia. Despite the fact that the disease can be disfiguring, scarring alopecia has rarely been described in this disease. Here, we present a 69‐year‐old woman who developed an erythrodermic episode of PRP associated with telogen effluvium that left an area of persistent alopecia of the scalp and resulted in hair loss in the eyebrows. The biopsy of that area of the scalp demonstrated a scarring alopecia with lichen‐planopilaris‐like features. Despite this histopathology, the alopecia responded well to treatment. This finding expands the context in which lichen planopilaris features can be found and demonstrates their good prognosis under early treatment.     

Acute cutaneous graft‐versus‐host disease resembling type II (atypical adult) pityriasis rubra pilaris

We present a case of cutaneous acute graft‐versus‐host disease (aGVHD) with confluent erythematous perifollicular hyperkeratosis and ichthyosiform scale in the clinical pattern of type II (atypical adult) pityriasis rubra pilaris (PRP), which developed 26 days after allogeneic peripheral blood stem cell transplant. Skin histology confirmed features of both aGVHD and PRP. The skin lesions were refractory to oral prednisolone and cyclosporine and only partially responsive to a combination of i.v. methylprednisolone, oral tacrolimus, oral mycophenolate mofetil, and infusions of anti‐thymocyte globulin and the tumour necrosis factor‐α inhibitor, etanercept.     

CARD14 Glu138 mutation in familial pityriasis rubra pilaris does not warrant differentiation from familial psoriasis

Some familial cases of pityriasis rubra pilaris (PRP) have the CARD14 gene mutations that are also detected in familial psoriasis vulgaris. However, genotype–phenotype correlation in these two entities is poorly understood. Here, we report a case of PRP with a new mutation in CARD14. Genomic analysis of a 40‐year‐old female patient with sporadic PRP type V identified a heterozygous dominant c.412G>A mutation (p.Glu138Lys) in CARD14. Two types of CARD14 mutations causing Glu138 substitutions have been reported in cases of familial PRP and pustular psoriasis. All three types, including the present case, are predicted to cause similar loss of the negative charges at this site. This suggests that the difference in molecular charge and the resulting change in molecular interaction around the N‐terminal end of the coiled‐coil region of CARD14 molecule do not determine the phenotypic differences between psoriasis and PRP.     

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 ×), diffuse effluvium (1 ×), and stenosis of the outer ear canal (1 ×). 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‐α antagonists are an effective treatment option for difficult cases.     

Case of pityriasis rubra pilaris progressed to generalized erythroderma following blockade of interleukin‐17A,but improved after blockade of interleukin‐12/23 p40

We report herein a case of a 72‐year‐old man with pityriasis rubra pilaris (PRP) that was refractory to conventional therapies. His skin lesions progressed to generalized erythroderma despite anti‐interleukin (IL)‐17A antibody therapy. Topical corticosteroids, emollients, systemic retinoid, methotrexate, cyclosporin and phototherapy yielded no therapeutic response. However, blockade of IL‐12/23 p40 dramatically improved his cutaneous lesions. Complete remission was achieved 4 weeks after the first injection of ustekinumab and maintained for more than 48 weeks. Our data indicate that IL‐12 was associated with the onset of PRP in this patient, rather than IL‐23. IL‐12 is critical for the differentiation of T‐helper (Th)1 cells. Thus, the Th1 pathway may be associated with the onset of PRP.     

Case of coincident severe acne and psoriasis in AIDS patient successfully treated with antiretroviral therapy

Cutaneous disorders remain a major problem in HIV‐infected patients, even under antiretroviral therapy (ART). Patients at any stage of HIV/AIDS may suffer from skin lesions. Acnes and psoriasis are both common chronic and inflammatory skin diseases, and the treatment becomes more challenging and complex when combined with HIV infection. Whether the incidence and severity of acne and psoriasis are related to HIV infection is still controversial. Here, we report a rare case of an AIDS patient who developed severe acne along with psoriasis. The patient had initially received multiple systemic and topical antipsoriatic and anti‐acne treatments which failed. Ultimately, he achieved dramatic clinical improvement after initiation of ART for main treatment. An 8‐year follow up demonstrated that the patient has been free of symptoms of both psoriasis and acne till now.     

Alopecia in association with severe seborrhoeic dermatitis following combination antiretroviral therapy for acute retroviral syndrome

We present a case where alopecia occurred with severe seborrhoeic dermatitis associated with the commencement of combination antiretroviral therapy for acute retroviral syndrome. We postulate that the eruption could represent a novel manifestation in association with immunological response to antiretroviral therapy.