Mucosal vulval lichen planus: outcome, clinical and laboratory features

BACKGROUND: Mucosal lichen planus of the vulva is a rare but increasingly recognized condition. It has potentially severe complications such as fusion of the labia and vagina; the risk of developing squamous cell carcinoma (SCC) may be increased. An association between hepatitis B and C infection and skin or oral lichen planus appears to exist in certain geographical areas. OBJECTIVE: To investigate the course of mucosal vulval lichen planus, its response to treatment and associated laboratory features. SUBJECTS AND METHODS: Forty-four women with mucosal vulval lichen planus were studied between 1997 and 2000 and laboratory data were collected. RESULTS: Thirty of 44 patients had additional oral lesions, only nine had cutaneous findings compatible with lichen planus. We did not find an association with antibodies to hepatitis B or C virus in this British study population. All women were treated with potent to very potent topical corticosteroids; however, in the majority of patients symptoms persisted. In seven (16%) patients vulval lichen planus was in remission after a disease duration between 2 and 18 years (mean 10.6 years). One patient developed a vulval SCC. CONCLUSIONS: Screening for hepatitis B and C in women with mucosal vulval lichen planus in the UK seems unnecessary. We recommend long-term follow-up, and that all non-healing ulcerative and papular lesions should be biopsied.     

Cervical epithelial abnormalities among women with vulval warts—no more common than among controls

Eighty-four women with vulval warts and 46 controls were examined for cervical epithelial abnormalities by cytology and colposcopy. The prevalence of colposcopically detected abnormalities was the same in the two groups. Minor cytologic abnormalities (less than moderate dyskaryosis) were more common among women with warts than among controls. It is concluded that women with vulval warts are no more likely than controls to have evidence of cervical epithelial abnormalities. These findings are discussed in the light of apparently contradictory findings by other workers.     

A child with localized vulval pemphigoid and IgG autoantibodies targeting the C-terminus of collagen XVII/BP180

Localized vulval pemphigoid of childhood (LVPC) has previously been reported in six girls. Clinical features and immunopathological data have suggested it to be a morphological variant of bullous pemphigoid. Epitope targets of the autoantibodies of these patients have not been defined in detail. We describe a 9-year-old girl with possible cicatricial LVPC and circulating IgG antibodies directed against native collagen XVII/BP180, its 120-kDa soluble ectodomain and against the C-terminus of collagen XVII/BP180. No reactivity was detected towards the NC16A domain of collagen XVII/BP180. Linear IgG and C3 deposits were found along the cutaneous basement membrane zone. On 1 mol/L salt-split skin, IgG autoantibodies were shown to bind to the epidermis, and the HLA type II allele DQB1*0301, a marker with significantly increased occurrence in patients with ocular and oral cicatricial pemphigoid, was identified in this patient. Our data suggest that LVPC is a variant of bullous pemphigoid in which direct immunofluorescence microscopy combined with immunoblot analysis can deliver valuable diagnostic information for differential diagnosis. However, differentiation between the scarring and non-scarring course of the disease cannot be made with the present diagnostic markers and therefore careful follow-up of patients with LVPC is required.     

TRPM7在胆管癌组织中的表达及其与预后的关系

目的：探讨瞬时受体电位离子通道(TRPM7)在胆管癌组织中的表达及其与预后的相关性。方法应用免疫组织化学 SP 法检测 TRPM7在49例胆管癌、7例胆管良性病变组织和36例癌旁正常胆管组织的表达情况,分析其与胆管癌临床病理的关系。结果胆管癌组织中 TRPM7表达阳性率为77.6%(38/49),高于其在胆管良性病变组织的表达阳性率(0,0/7)及癌旁正常胆管组织中的表达阳性率(2.8%,1/36),差异均有统计学意义(P <0.05)。TRPM7蛋白的表达与胆管癌的 TNM 分期、淋巴结转移和脏器转移相关(P <0.05),但与患者的年龄、性别、部位、肿瘤分化程度及肝炎无相关性(P >0.05)。Kaplan-Meier 生存分析发现 TRPM7阳性表达患者的生存期低于 TRPM7阴性表达患者(P <0.05)。Cox 多因素分析显示,TRPM7的表达水平与生存预后显著相关(P <0.05),是影响预后的独立危险因素。结论TRPM7在胆管癌的发生、发展和浸润转移中发挥着重要作用,是影响胆管癌患者预后的重要因素。     

Topical tacrolimus in the management of lichen sclerosus

The effective management of vulval lichen sclerosus (LS) currently depends upon the use of topical steroids and emollients. There are concerns with regard to the long-term toxicity of potent steroids and therefore is a need to consider effective alternatives. Immunomodulatory macrolactams offer an alternative to steroids in the management of some other inflammatory skin disorders and it would seem reasonable therefore to assess their activity in LS. This pilot study of 16 histologically confirmed cases of LS suggests that macrolactams have a positive pharmacological effect.     

Single-episode photodynamic therapy and vulval intraepithelial neoplasia type III resistant to conventional therapy

BACKGROUND: Photodynamic therapy (PDT) using topical 5-aminolaevulinic acid (5-ALA) has been suggested as an effective and tissue-conserving method of treating carcinoma in situ of the vulva. OBJECTIVES: To evaluate PDT in patients with vulval intraepithelial neoplasia type III (VIN III). METHODS: Topical PDT was performed in six patients with VIN III. Five of the six patients had persistent disease following treatment with other modalities including 5-fluorouracil cream, cryotherapy, carbon dioxide laser ablation and excision. Each patient was treated once with a fluence of 150 J cm-2 using a broad-band light source (580-740 nm) 4 h after topical application of 20% 5-ALA. Patients were reviewed clinically at 1 month and 6 months after treatment. RESULTS: All of the patients developed initial erythema of treated sites, three with subsequent erosions. All patients had clinically evident persistent VIN III at 1-month review. Five patients have subsequently undergone surgical treatment and one is regularly reviewed. CONCLUSIONS: This small uncontrolled study indicates that, as currently administered, a single episode of topical PDT is not effective in the management of treatment-resistant VIN III.     

Photodynamic therapy using meta-tetrahydroxyphenylchlorin (Foscan) for the treatment of vulval intraepithelial neoplasia

BACKGROUND: Photodynamic therapy (PDT) has unique properties which make it suitable for the local treatment of superficial epithelial disorders; it has been suggested as a useful treatment for carcinoma in situ of the vulva. OBJECTIVES: To evaluate the effect of the systemic photosensitizing agent meta-tetrahydroxyphenylchlorin (mTHPC or temoporfin; Foscan, Biolitec, Edinburgh, U.K.) in vulval intraepithelial neoplasia type III (VIN III). METHODS: PDT using mTHPC was performed in six patients with VIN III. A dose of 0.1 mg kg(-1) body weight mTHPC was injected intravenously and the area of VIN irradiated 96 h later with 652-nm light from a diode laser. Patients were reviewed 1 week, 6 months and 2 years following treatment. RESULTS: Patients experienced only minimal pain from the initial treatment but two patients subsequently developed severe pain at the treated site for up to 2 weeks following PDT. All patients developed oedema and slough formation at the treated site and one patient developed cellulitis. At 6 months two patients had developed small recurrences of VIN at the original site and one patient had an area of VIN at a new site. These were treated either with further PDT or with a small excision. At 2 years there was no recurrence of VIN at the original site in all patients reviewed. CONCLUSIONS: This small case series demonstrates that mTHPC-PDT is a useful initial treatment for VIN III. It is relatively selective, shows good cosmesis and conserves form and function. This is a major advantage over surgery. Repeat treatments are also possible, which is important in a condition such as VIN, which tends to be multifocal. Systemic mTHPC-PDT appears to have an advantage over topical 5-aminolaevulinic acid-PDT as the photosensitizer is distributed widely in areas of disease and consequently identifies foci which may not be apparent clinically but become evident when illuminated.