Rapid resolution of primary vulval adult Langerhans cell histiocytosis with very potent topical corticosteroids

Langerhans cell histiocytosis is a rare idiopathic disorder with protean clinical presentations. Primary unifocal single-system disease of the vulva is even less common. We report a 62-year-old female patient presenting with an 18-month history of pruritus and burning of the vulva. Clinical examination of the vulva showed a tender nodule of the right labium minus. Histology confirmed Langerhans cell histiocytosis. Systemic involvement was excluded. Within 1 month the use of clobetasol propionate ointment led to resolution of both the patient's symptoms and the clinical appearance of the affected right labium minus. This resolution was maintained 12 months later.     

The treatment of vulval lichen sclerosus in prepubertal girls: a critically appraised topic

Lichen sclerosus is one of the dermatoses that specifically affects the anogenital skin. It has peaks of incidence in prepubertal girls and postmenopausal women. The objective of this critical appraisal was to review systematically the evidence for efficacy and safety of different treatments. There are no randomized controlled studies of treatment in prepubertal girls and most studies are small case series or case reports. There is little focus on quality of life.     

T cells reactive with the NC16A domain of BP180 are present in vulval lichen sclerosus and lichen planus

Background Lichen sclerosus (LS) is a chronic inflammatory skin condition. The recent demonstration of circulating autoantibodies to extracellular matrix protein 1 and to basement membrane zone (BMZ) components, chiefly BP180, suggests that autoimmunity to these components might contribute to pathogenesis. However, there is no binding of autoantibodies in vivo and as LS is characterized by a lymphocytic infiltrate, it seems likely that LS is mediated, in part, by antigen‐specific lymphocytes. Similar mechanisms may apply to vulval lichen planus (LP), an interface dermatitis, with clinical and immunological overlap with LS. Objectives This study aims to test the hypothesis that T cells reactive with the NC16A domain of BP180 are present in the peripheral blood of patients with vulval LS and LP. Methods Isolated peripheral blood mononuclear cells from 14 patients with vulval LS, 5 with vulval LP and 4 healthy controls were grown in vitro. We examined for immunogenicity of overlapping peptides spanning the NC16A domain of BP180 using interferon‐γ enzyme‐linked immunospot assay (ELIspot) on the cultured T‐cell lines. BMZ antibodies were assayed, HLA type determined and clinical parameters noted. Results Significant interferon‐γ production was observed in response to the NC16A peptides in 6 of the 14 vulval LS and 2 of the 5 LP patients, but not in the control subjects. There was an associated autoantibody response to BP180 in 3 LS and 1 LP patient with T‐cell responses. These data suggest that in some vulval LS and LP patients, NC16A domain‐specific T cells circulate at sufficiently high frequency to be detectable in vitro and show rapid effector function. There was no association with HLA type or clinical parameters. Conclusion We have demonstrated that in > 40% of our vulval LS and LP patients, the NC16A domain of BP180 is a target for circulating T cells, and in vulval LS and LP there are associated autoantibodies to BP180.     

Extragenital bullous lichen sclerosus: A case report and literature review

Lichen sclerosus (LS) is a chronic dermatosis characterized by atrophic white papules or plaques, most commonly occurring on the anogenital skin. Blisters have been rarely described developing in the background of extragenital LS. A 74-year-old woman with a 4-year history of sclerotic patches on the trunk showed a flaccid bulla on the lower back for 3 months. The histopathological finding of the skin biopsy was consistent with the diagnosis of bullous LS. In this paper, we present this uncommon case, review the literature on extragenital bullous LS, discuss the pathogenesis, and provide some treatment options for the case.     

A new delivery system of clobetasol-17-propionate (lipid-loaded microspheres 0.025%) compared with a conventional formulation (lipophilic ointment in a hydrophilic phase 0.025%) in topical treatment of atrophic/erosive oral lichen planus. A Phase IV, randomized, observer-blinded, parallel group clinical trial

BACKGROUND: Topical application of clobetasol-17-propionate has been diffusely reported as an efficacious therapy in atrophic/erosive oral lichen planus (OLP), without exposing the patient to systemic side-effects. However, prolonged contact and respective topical effects on the oral mucosa should be avoided. OBJECTIVES: The aim of the present study was to evaluate efficacy and compliance of new lipid microspheres loaded with 0.025% of clobetasol propionate (formulation A) compared with a commonly used formulation (a sort of dispersion of a lipophilic ointment in a hydrophilic phase) with the same amount of drug (formulation B) in the topical treatment of OLP. PATIENTS AND METHODS: Fifty patients with symptomatic OLP were enrolled in a controlled single-blind phase IV clinical trial. After a dropout of five patients, a total of 45 patients [12 males and 33 females; mean age 61.1 years (+/- 12.3 SD; range 25-82)] were treated (17 with formulation A and 28 with formulation B, matched for gender and age; P > 0.2) with the same dosage regimen. At times T0, T1 and T2 we evaluated the following parameters: (i) pain score (by linear visual analogue scale; 0-100); (ii) clinical score; (iii) clinical resolution; and (iv) patient compliance. Statistical analysis was calculated using S-Plus 4.0 and SPSS 9.0 (Student's t-test, chi(2), Kolmogorov-Smirnow, Friedman, Student-Newman-Keuls, Mann-Whitney U-test and Spearman tests). RESULTS: Both formulations were found to be similar for parameters ii, iii and iv, although with a better general trend for formulation A; a significant difference was registered for formulation A in terms of a reduction in painful symptoms (parameter i) at time T2 (P = 0.02). CONCLUSIONS: Our results suggest that the new topical drug delivery system (formulation A) may enhance, at least in terms of symptom remission and compliance, the effectiveness of clobetasol propionate at a dose of 0.025% in OLP therapy.     

复方丙酸氯倍他索软膏治疗寻常性银屑病临床疗效观察

目的:观察复方丙酸氯倍他索软膏治疗寻常性银屑病的疗效和安全性。方法:将入组的360例寻常性银屑病患者分为治疗组(复方丙酸氯倍他索软膏组120例)、对照1组(0.02%丙酸氯倍他索霜组120例)和对照2组(0.05%全反式维A酸霜组120例),采用多中心、随机双盲、平行对照的方法对寻常性银屑病患者连续用药4周。结果:3组患者的痊愈率分别为48.33%、18.33%和3.48%,有效率分别为92.50%、50.83%和31.30%。组间比较(试验组与对照1组比较,试验组与对照2组比较)显示,痊愈率和有效率差异均有显著性(P<0.01)。试验组和对照1组的不良反应发生率较轻微,对照2组不良反应发生率为11.67%,有3例因不良反应较重中途停止治疗。结论:复方丙酸氯倍他索软膏是一种安全有效的治疗寻常性银屑病的外用药,其疗效优于单用丙酸氯倍他索霜或全反式维A酸霜。     

HPA‐suppressive effects of aqueous clobetasol propionate in the treatment of patients with oral lichen planus

Background Oral topical corticosteroids have potential to produce inhibition of the hypothalamus–pituitary–adrenal (HPA) axis. Objective To assess whether clobetasol propionate (CP) in aqueous solution causes HPA inhibition. Patients and methods Sixty‐two patients with oral lichen planus or oral lichenoid lesions presenting with severe lesions were treated with topical oral 0.05% CP plus 100 000 IU/cm³ nystatin in aqueous solution. Initial treatment of three 5‐min mouthwashes (10 mL) daily was reduced, when the response was deemed complete or excellent, to a maintenance treatment of one 5‐min mouthwash on alternate days for 6 months; treatment was then withdrawn and patients were followed up for 1 year. HPA function was assessed by plasma cortisol measurement and adrenocorticotropin (ACTH) stimulation at the end of the initial and maintenance treatment regimens. Results The HPA axis was more frequently inhibited during initial (53/62; 85.5%) vs. maintenance (2/49; 4%) regimens of aqueous CP. Limitations In patients with morning plasma cortisol levels between 3 and 18 μg/dL, a normal result for the ACTH stimulation test only moderately reduces the possibility that a patient has secondary adrenal insufficiency. This can be considered a minor limitation in our study, as only three patients required additional assessment with the ACTH stimulation test. Conclusions Hypothalamus–pituitary–adrenal inhibition is substantial during initial treatment with aqueous CP three times daily.     

Lichen sclerosus with enlarged vessels: A variant of lichen sclerosus in young girls

Vascular findings have rarely been described in the setting of lichen sclerosus. Enlarged vessels within the atrophic plaques have been observed on the free margins of the labia minora and clitoral hood. The enlarged vessels completely remit upon treatment of lichen sclerosus with an ultra‐potent corticosteroid ointment in the acute phase. During maintenance therapy with calcineurin inhibitors, there was no recurrence of the enlarged vessels.     

硬化性苔藓64例临床及病理学特征分析

回顾性分析我院皮肤科2016年1月1日至2020年6月30日经组织病理学确诊的64例硬化性苔藓(LS)患者的临床及病理资料,平均年龄(36.84±17.84)岁;男女比例1∶2.5。外阴组患者25例(39%),非外阴组39例(61%),两组性别、年龄差异无统计学意义。非外阴组患者临床误诊率(38.5%)高于外阴组(12%),差异有统计学意义(P=0.022);外阴组患者比非外阴组患者更易出现瘙痒,组织病理学中更容易出现表皮增生、嗜酸粒细胞浸润及毛细血管扩张,两组比较差异有统计学意义(P值分别为0.048、<0.001、0.021及0.035)。9例患者行皮肤镜检查,镜下均见黄白色无结构区及毛细血管扩张,6例见毛囊角栓,7例见亮白色条束。本研究显示非外阴部位的LS并不少见,且更容易出现误诊,皮肤镜检查具有一定辅助诊断作用。     

Treatment of nail psoriasis with 8% clobetasol nail lacquer: positive experience in 10 patients

BACKGROUND: Treatment of nail psoriasis is difficult. Several topical therapies have been employed with poor results because drug penetration is limited in this localization. Recently, a new formulation containing 8% clobetasol-17-propionate in a colourless nail lacquer vehicle has shown good results in the control of nail psoriasis. OBJECTIVE: To determine the efficacy and safety of 8% clobetasol-17-propionate in a lacquer vehicle in nail psoriasis. METHODS: Ten patients with both nail bed and matrix psoriasis were included in the study. They were treated with a colourless nail lacquer containing 8% clobetasol-17-propionate that was applied once daily for 21 days and then twice weekly for 9 months. RESULTS: Within 4 weeks of therapy there was a reduction of all the nail alterations, including nail pain. Therapeutic response was directly related to the length of therapy. The nail parameters that responded best to therapy were onycholysis, pitting and salmon patches. Subungual hyperkeratosis and splinter haemorrhages on the other hand had moderate and poor improvement, respectively. The treatment was well tolerated in all of the patients and there were no local (i.e. atrophy and sobreinfection) or systemic secondary effects. CONCLUSIONS: The formulation containing 8% clobetasol-17-propionate is a safe, effective and cosmetically highly acceptable treatment for nail bed and matrix psoriasis.     

Familial lichen sclerosus et atrophicus in association with CREST syndrome: a case report

Lichen sclerosus et atrophicus is an uncommon disease which appears to be multifaetorial in aetiology. We describe a case of a young woman with CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangiectasial who has a documented family history of two sisters with lichen sclerosus et atrophicus. She presented with vulvar pruritas in association with dyspareunia. and biopsy of atrophic white vulvar lesions was consistent with lichen sclerosus et atrophicus. Lichen sclerosus et atrophicus has been previously noted to occur in association with morphoea and lichen planus, although it has never been reported in conjunction with CREST syndrome.     

Treatment of scalp psoriasis with clobetasol‐17 propionate 0.05% shampoo: a study on daily clinical practice

Background Safety and clinical effectiveness of clobetasol‐17 propionate 0.05% shampoo have been shown in patients with scalp psoriasis. Aim First, to evaluate treatment satisfaction, user convenience safety and effectiveness of clobetasol‐17 propionate 0.05% shampoo treatment in daily clinical practice. Second, to identify subgroup variables that may predict treatment success or failure. Methods A total of 56 patients with scalp psoriasis were treated with short‐contact clobetasol‐17 propionate 0.05% shampoo once daily for 4 weeks. Data on treatment satisfaction, user convenience, safety and effectiveness were assessed on a 7‐point Likert scale using postal questionnaires. Subgroup analyses were performed to identify variables that may predict treatment outcome. Results A total of 41 patients returned both questionnaires (73%). Positive treatment satisfaction and user convenience were reported by 66% and 79% of patients respectively. Patient‐rated indicators for disease severity improved by 39–46% (P < 0.05%). No major side‐effects were reported. Subgroup analyses did not reveal any statistically significant patient variable that may predict treatment outcome. However, a tendency towards improved treatment satisfaction was observed in patients who had received fewer topical antipsoriatic treatments previously (P > 0.05). Conclusions Short‐contact treatment with clobetasol‐17 propionate 0.05% shampoo has high user convenience and patient satisfaction rates. Moreover, the treatment is well‐tolerated and efficacious from patients’ perspective. Subgroup analyses did not reveal factors predicting treatment outcome, although treatment success tended to be more evident in patients who had received fewer treatments previously.