Management of anogenital lichen sclerosus

Lichen sclerosus (LS) is a skin condition that affects genital and extra genital epithelia in both males and females of all ages and it may occur in association with other autoimmune disease. Currently, the first line effective treatment is an ultra-potent topical corticosteroid. The long-term sequelae of LS include scarring, malignancy, which is rare, and psychosexual disfunction, which is common.     

Evidence for Borrelia burgdorferi in morphea and lichen sclerosus

BACKGROUND: Borrelia burgdorferi (Bb) infection has been implicated in the development of morphea and lichen sclerosus; however, conflicting results have been reported with different investigational methods from different regions. We looked for evidence of Bb in patients with morphea and lichen sclerosus by polymerase chain reaction (PCR) analysis of of skin biopsy samples. METHODS: Formalin-fixed, paraffin-embedded skin biopsy samples from 10 patients with morphea and 12 patients with lichen sclerosus were investigated by PCR analysis for the presence of Bb. RESULTS: The presence of Bb DNA was demonstrated in three of 10 patients with morphea and six of 12 patients with lichen sclerosus by nested PCR. CONCLUSIONS: The data obtained in this study suggest that Bb may play a role in the etiopathogenesis of both morphea and lichen sclerosus at least in the western parts of Turkey.     

Successful treatment of anogenital lichen sclerosus with topical tacrolimus

BACKGROUND: Lichen sclerosus of the anogenital area is a chronic inflammatory and fibrosclerotic disease associated with substantial morbidity. Topical ultrapotent corticosteroids are currently the treatment of choice. OBSERVATIONS: Three prepubertal girls and 3 adults (2 men, 1 woman) were treated with 0.1% tacrolimus ointment once daily. All patients experienced complete resolution with long-lasting remission for up to 1 year. No major adverse effects were observed, and treatment was well tolerated. CONCLUSIONS: Topical tacrolimus is a promising novel agent in the treatment of lichen sclerosus of the anogenital area. A major advantage over topical corticosteroids is the lack of skin atrophy. Further clinical trials are warranted to confirm our findings.     

Pimecrolimus 1% cream for anogenital lichen sclerosus in childhood

Background

Lichen sclerosus is a chronic inflammatory disease with a predilection of the anogenital region. Because of the potential side effects of repeated local application of potent glucocorticosteroids, equally-effective, safer therapeutic options are required, especially in the treatment of children.

Case presentations

We report on the efficacy of twice-daily application of pimecrolimus 1% cream in four prepubertal girls (range of age: 4 to 9 years) who suffered from anogenital lichen sclerosus. After three to four-month treatment, all patients had almost complete clinical remission including relief from itch, pain and inflammation. Only minor improvement was observed for the white sclerotic lesions. No significant side effects have been observed.

Conclusions

Topical pimecrolimus appears to be an effective and safe treatment for children with anogenital lichen sclerosus. The clinical benefits observed in the four patient presented particularly include relief of pruritus, pain and inflammation. Vehicle-controlled studies on a larger number of patients are now warranted to substantiate our promising findings, and to investigate long-term efficacy and safety of topical pimecrolimus in anogenital lichen sclerosus.

     

Concomitance of lichen sclerosus et atrophicus and morphea in the same lesions

A 65-year-old woman presented atrophic, slightly indurated plaques on the trunk and a sclerotic, hemorragic plaque on the right forearm. In three biopsies, diagnostic features of both lichen selerosus et atrophicus (LSA) and morphea coexisted. Elastic fibers were absent in the papillary dermis and present in the sclerotic reticular dermis. Although many patients with lesions of both morphea and LSA have been reported, the coexistence of the two histological features in the same lesion is less frequent.     

Histological evidence for spirochetal origin of morphea and lichen sclerosus et atrophicans

In order to elucidate the possible spirochetal origin of morphea and lichen sclerosus et atrophicans (LSA), we investigated biopsy specimens from 13 patients with morphea and 13 patients with LSA. Four patients with acrodermatitis chronica atrophicans (ACA), three with erythema chronicum migrans (ECM), and 11 patients with other inflammatory dermatoses served as controls. Formalin-fixed, paraffin-embedded sections were stained by an avidin-biotin-immunoperoxidase method, using serum of a patient with ACA that contained IgG antibodies to Borrelia burgdorferi. As positive control substrate, formalin-fixed culture suspensions of B. burgdorferi strain B31 were used. They presented either as thin, mostly linear, but partially dotted, stained spirochetes or, in contrast, as thick, "swollen," heavily stained organisms. Identical structures could also be detected on histological sections of one patient with ECM, four patients with morphea, and six patients with LSA. These findings provide evidence for the spirochetal origin of both morphea and LSA.     

Diffuse fasciitis with eosinophilia associated with morphea and lichen sclerosus et atrophicus

Diffuse fasciitis with eosinophilia (DFE) is a well described syndrome, among the connective tissue disorders. However, DFE is not commonly accepted as an own entity, because of its rare evolvement in scleroderma and the often indistinguishable histological pattern of both diseases. Here we report on the association of DFE and morphea in two patients and an additional lichen sclerosus in one of them. This points to a close relationship of DFE and other connective tissue diseases.     

Spirochetal forms in the dermal lesions of morphea and lichen sclerosus et atrophicus

Morphea and lichen sclerosus et atrophicus are cutaneous diseases that are manifest by an early edematous stage, followed later by sclerosis and atrophy. They share features with acrodermatitis chronica atrophicans and erythema chronicum migrans, diseases that have been linked to infection by the spirochete Borrelia burgdorferi. A modified silver stain was used to identify the presence of spirochetes in skin biopsy specimens of patients with morphea and lichen sclerosus et atrophicus. Spirochetal forms were identified in the lesional skin of 10 of 25 patients with morphea and in 10 of 21 cases of lichen sclerosus et atrophicus. These spiral forms of bacteria had a significant tendency to occur in early and fully developed lesions of morphea and in early lesions of lichen sclerosus et atrophicus, whereas they tended to be absent in lesions demonstrating late pathological changes.     

Linear morphea with overlying lichen sclerosus and calcinosis cutis associated with juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is associated with many distinguishing features including cutaneous calcinosis, vasculitis, and ulcerated lesions. In this case, we describe an unusual presentation in a 12-year-old girl who had muscle weakness along with linear morphea over the right upper and lower extremities with overlying lichen sclerosus and calcinosis cutis. Of interest, these initial cutaneous manifestations occurred years before onset of myositis.     

Alterations of basement membrane zone and cutaneous microvasculature in morphea and extragenital lichen sclerosus

The aim of this study was to compare alterations of the basement membrane zone (BMZ) and to visualize changes within the skin vascular network in morphea and extragenital lichen sclerosus with the use of laser scanning confocal microscopy. This work was performed in eight plaques of morphea (three active and five inactive) and eight of lichen sclerosus (three of short duration and five long-lasting). Biopsy specimens from six healthy individuals served as controls. The biopsies were cut into 40-microm-thick sections, labeled with antibodies against beta4-intergin (a lamina lucida marker), collagen IV, and the N-terminal end of collagen VII (lamina densa markers) and C-terminal end of collagen VII (a sublamina densa marker) and studied using laser scanning confocal microscopy. Three-dimensional reconstruction of various regions of the BMZ showed a decreased number and size of the dermal papillae both in morphea and lichen sclerosus compared with normal skin. In morphea, the continuity of the BMZ at the level of lamina lucida, lamina densa, and sublamina densa was preserved whereas in LS numerous invaginations and holes were present in the BMZ at the level of the lamina lucida and lamina densa. Thus the alterations of the BMZ in morphea differ from those in lichen sclerosus. Three-dimensional reconstruction of the skin vascular network showed increased angiogenesis only in the early inflammatory stage of morphea, whereas in inactive morphea and lichen sclerosus various numbers of enlarged vessels were visible. The changes in the vascular network in morphea appear to be related to the activity of the disease.     

硬化性苔藓合并扁平苔藓、硬斑病一例并文献复习

硬化性苔藓(LS),扁平苔藓(LP)和硬斑病是三种病因不明的皮肤病,已有多篇任意两种疾病合并的报道,但患者同患LS、LP和硬斑病少见,目前未见国内有相关报道。本文报道1例LS合并LP和硬斑病,并对国外已报道的7例病例进行回顾性分析。结果示8例患者中男2例,女6例,平均年龄(60.3±15.1)岁。8例患者的LS与硬斑病同时发生或硬斑病发病早于LS,同一皮损的病理切片同时具有LS与硬斑病的特征,LP发病可早于或晚于LS和硬斑病;8例患者皮损主要表现为生殖器外LS、泛发型硬斑病、经典或糜烂型LP;6例有免疫相关异常指标,4例合并其他自身免疫性疾病。     

Vulval lichen sclerosus and lichen planus

Lichen sclerosus (LS) and lichen planus (LP) are both immunologically mediated diseases with a preference for the genitalia. The basic principles of management of vulval LS and vulvovaginal LP are the same and involve explanation of the disease, emphasizing the chronic nature of the condition and outlining treatment options. The main difference between the two conditions is that LP has a propensity to involve the mucous membranes including the mouth and vagina which are rarely affected in LS. First‐line treatment for LS is a super‐potent topical corticosteroid ointment which has a high response rate. Erosive vulvovaginal LP is more challenging to treat. Second‐line therapies include topical calcineurin inhibitors and systemic agents. There is limited evidence for systemic treatments for both conditions. The risk of vulval squamous cell carcinoma (SCC) is increased in both LP and LS, and it is not known how treatment affects this risk. We recommend teaching self‐examination and longitudinal evaluation.     

The period prevalence of oral lichen planus in a cohort of patients with vulvar lichen sclerosus

Background Lichen sclerosus and lichen planus are chronic inflammatory mucocutaneous disorders that may coexist. Objective The aim of this study was to estimate the period prevalence of oral lichen planus in a cohort of patients with vulvar lichen sclerosus and to document their clinical characteristics. Methods We report a series of cases of vulvar lichen sclerosus presenting to two dermatologist‐led vulvar clinics in Oxfordshire, England between 1997 and 2007 with coexistent clinical signs of oral lichen planus. Results Thirteen cases with coexistent vulvar lichen sclerosus and oral lichen planus were identified, of which five had oral biopsies. Four oral biopsies showed histological features consistent with lichen planus. One oral biopsy was not diagnostic but compatible with oral lichen planus. No cases of oral lichen sclerosus were identified. The period prevalence of oral lichen planus was 6 per 1000 cases of vulvar lichen sclerosus. Conclusion The period prevalence of oral lichen planus in women with vulvar lichen sclerosus (0.6%) is similar to that reported for oral lichen planus in the general population (1–2%).