Diagnostic criteria, severity classification and guidelines of lichen sclerosus et atrophicus.

We established diagnostic criteria and severity classification of lichen sclerosus et atrophicus, because there is no established diagnostic criteria or widely accepted severity classification of the disease. Also, there is no clinical guideline for lichen sclerosus et atrophicus in Japan, so we proposed its clinical guideline. The clinical guidelines were formulated by clinical questions and recommendations on the basis of evidence-based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1.0). We aimed to make the guidelines easy to use and reliable including the newest evidence, and to present guidance for various clinical problems in treatment of lichen sclerosus et atrophicus.

O tratamento bem-sucedido com fototerapia UVB banda estreita em um caso raro de líquen escleroso extragenital generalizado / Successful treatment of a rare case of widespread extragenital lichen sclerosus with narrow band UVB phototherapy

O líquen escleroso é patologia incomum acometendo tanto região genital quanto extragenital, constituindo apresentação atípica da doença. O uso de corticosteroides tópicos é considerado primeira linha para tratamento da doença genital. Lesões extragenitais são menos sensíveis a essa terapêutica. Relata-se caso de líquen escleroso em paciente do sexo feminino, de 82 anos, com envolvimento extragenital generalizado e tratamento bem-sucedido com fototerapia UVB de banda estreita. O líquen escleroso é dermatose inflamatória crônica, e as formas disseminadas da doença são raras e sem consenso em relação ao tratamento da forma extragenital. A opção pela terapia com UVB de banda estreita baseou-se nos relatos da literatura.

Lichen sclerosus is an uncommon condition affecting both the genital and extragenital region, the latter constituting an atypical occurrence of the disorder. The use of topical corticosteroids is considered the first-line treatment of this genital disease. Extragenital lesions are less sensitive to this therapy. The present study describes a case of lichen sclerosus in an 82-year-old female patient, with widespread extragenital involvement and successful treatment with narrow band UVB phototherapy. Lichen sclerosus is a chronic inflammatory dermatosis and disseminated forms of the condition are rare, with an absence of consensus on the treatment of the extragenital form. The choice for narrow band UVB therapy was based on the literature.

[Lichen sclerosus--clinical and therapeutic aspects].

Lichen sclerosus (LS) is a lymphocyte-mediated inflammatory dermatosis with a characteristic location (85-98%) in the anogenital region. The authors point out the main features in the epidemiology and clinical presentation of the disease and the possible approach to neoplastic development. Expanded differential diagnosis of LS sparked not only dermatologists but also gynecologists, urologists and GPs. Points are the chronic course of the disease and resistance to therapy. Discuss the results of the treatment of LS with less potent topical corticosteroids, calcineurin inhibitors, phototherapy and photodynamic therapy, surgery.

Treatment regimens, protocols, dosage, and indications for UVA1 phototherapy: facts and controversies.

During the last three decades, ultraviolet A1 (UVA1) phototherapy has emerged as a specific phototherapeutic modality with distinct modes of action and some well established indications. Atopic dermatitis, localized scleroderma, and systemic lupus erythematosus seem to be the conditions with the best evidence regarding efficacy and safety of UVA1 phototherapy. Further indications for UVA1 include subacute prurigo, lichen sclerosus, dyshidrotic dermatitis, cutaneous T cell lymphoma, urticaria pigmentosa, and pityriasis rosea; nevertheless, there are some unknowns, uncertainties, and controversies concerning short- and long-term side effects, efficacy and dosage regimens of UVA1 phototherapy in some conditions. We describe and discuss treatment regimens, protocols, dosage, and indications for UVA1 phototherapy.

Diagnosis and treatment of lichen sclerosus: an update.

Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin. LS is a debilitating disease, causing itch, pain, dysuria and restriction of micturition, dyspareunia, and significant sexual dysfunction in women and men. Many findings obtained in recent years point more and more towards an autoimmune-induced disease in genetically predisposed patients and further away from an important impact of hormonal factors. Preceding infections may play a provocative part. The role for Borrelia is still controversial. Trauma and an occlusive moist environment may act as precipitating factors. Potent and ultrapotent topical corticosteroids still head the therapeutic armamentarium. Topical calcineurin inhibitors are discussed as alternatives in the treatment of LS in patients who have failed therapy with ultrapotent corticosteroids, or who have a contraindication for the use of corticosteroids. Topical and systemic retinoids may be useful in selected cases. Phototherapy for extragenital LS and photodynamic therapy for genital LS may be therapeutic options in rare cases refractory to the already mentioned treatment. Surgery is restricted to scarring processes leading to functional impairment. In men, circumcision is effective in the majority of cases, but recurrences are well described. Anogenital LS is associated with an increased risk for squamous cell carcinoma of the vulva or penis. This review updates the epidemiology, clinical presentation, histopathology, pathogenesis, and management of LS of the female and male genitals and extragenital LS in adults and children.

[Vulvar lichen sclerosus]. / Le lichen scléreux vulvaire.

Vulvar lichen sclerosus is a frequent mucocutaneous disease especially affecting 50 to 60 year-old women but with a possible onset at very young age. Symptoms are most disabling including pruritus and dyspareunia. Vulvar mucosa gradually becomes more white and atrophied. Degeneration into epidermoid carcinoma is possible. Treatment only consists of topical corticosteroids.

[Balneophotochemotherapy with 8-methoxypsoralen in lichen sclerosis et atrophicus]. / Balneophotochemotherapie mit 8-Methoxypsoralen bei Lichen sclerosus et atrophicus.

A 66-year-old woman with longstanding lichen sclerosus et atrophicus improved strikingly with PUVA bath photochemotherapy over a period of 6 weeks. The cumulative UVA dose was 31.7 J/cm2; the single UVA dose ranged from 0.3 to 2.3 J/cm2. After 16 treatment sessions, the sclerotic lesions had softened greatly, while after 24 treatments, the skin lesions were almost completely cleared and pruritus was diminished. Histopathological analysis of biopsy specimens from previously affected sites as well as 20 MHz ultrasound examinations showed almost no residual sclerosis. Although long-term results are not yet available, PUVA bath photochemotherapy seems to be a promising and effective new treatment modality without systemic side effects for patients with disseminated lichen sclerosus et atrophicus.