A pediatric case of generalized lichen aureus

Lichen aureus is a particularly rare subtype of pigmented purpuric dermatosis and is characterized by the sudden appearance of golden or rust‐colored macules or needle‐tip‐sized flat papules (concentrated in one region to form lichenoid papules) on the lower limbs. These skin lesions are usually confined to an isolated, unilateral distribution, and linear segmental distribution is rare. In this report, we have documented one such case, where the lesions on the limb were arranged in strips (segmental distribution) that roughly followed the direction of the venous drainage. And the first attack and subsequent aggravation were both associated with the onset of allergic rhinitis, a Type I hypersensitivity.     

Linear morphea overlying site of previous lichen aureus in a pediatric patient

Lichen aureus is a pigmented purpuric dermatosis that tends to occur on the lower extremities, and linear morphea is an inflammatory disease of the dermis and subcutaneous fat that leads to a scarlike induration. We present a pediatric case of linear morphea developing in the same anatomic distribution as prior lichen aureus. Due to the possible progression of lichen aureus to morphea described herein and the difficulty in diagnosing early morphea, close follow‐up may be considered for pediatric patients who present with this pigmented purpuric dermatosis.     

半胱天冬酶-3、Bcl-2在扁平苔藓皮损中的表达

目的：探讨半胱天冬酶（caspase）-3和Bcl-2在扁平苔藓（LP）中的表达及意义。方法：应用免疫组化SP法对30例LP患者皮损和10名正常对照者皮肤中半胱天冬酶-3和Bcl-2的表达进行检测。结果：半胱天冬酶-3在LP皮损表皮中表达明显增强．Bcl-2在LP皮损表皮、真皮淋巴细胞浸润带的表达均明显增强;正常对照者皮肤基底细胞中半胱天冬酶-3表达仅为20％,Bcl-2偶见表达。结论：半胱天冬酶-3和Bcl-2在皮肤LP发生和发展中起一定作用。     

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%).     

Antinuclear antibodies in patients with lichen planus

Abstract Lichen planus (LP) is a mucocutaneous syndrome of yet uncertain pathogenesis, and it has usually been considered to be a dermatosis without antinuclear antibodies (ANA) nor other specific auto-antibodies. Over 10 years ago a series of indirect immunofluorescence researches with patients' lesional skin and serum disclosed the presence of lichen planus specific antigens (LPSA). After this, a number of substrates have been submitted for evaluation. In this study we have carried out indirect immunofluorescent test in relation with different substrates, with the aim of verifying whether the negative results previously obtained were due to poor sensitivity of the substrates employed. Subsequently we have compared the results obtained in the erosive forms of LP with those obtained in the non-erosive forms. We have concluded that rat oesophagus is a better substrate for the detection of ANA in patients with LP, as it has proved to have a positivity rate of 40.42%. Monkey oesophagus has provided a rate of 27.6%, and HEP-2 cells and rat liver have proved to be unsuitable. In addition, we have observed that the frequency of ANA is higher in the erosive forms of LP (P= 0.0389). In this article we demonstrate that the presence of ANA in patients with LP depends on the substrate employed, the most suitable substrate being rat oesophagus. Also, we demonstrate that ANA is more frequently observed in patients with erosive LP.     

Evaluating and treating patients with oral lichen planus

Lichen planus (LP) is a relatively common disorder with a reported incidence approximating that of psoriasis. Unlike cutaneous lesions, which in the majority of instances are self-limiting, oral lesions are chronic and are a potential source of significant morbidity. Furthermore, compared to cutaneous lesions, oral lesions are more difficult to control and are often refractory to therapy. Patients with oral lichen planus (OLP) frequently have concomitant disease in one or more extraoral sites and therefore require a thorough evaluation to uncover all affected sites of involvement. Genital involvement, which develops in approximately 20% of women patients with OLP, and esophageal involvement may result in scarring if undetected and not treated appropriately. Since no therapy for OLP is curative, the primary goal for symptomatic patients is palliation. Relief can be achieved in the majority of patients with topical corticosteroids alone or in combination with other immunomodulatory topical agents. Infrequently patients require the prolonged use of systemic medications to control the disorder. All treatments are nonspecific and directed at eliminating inflammation, and therefore are only partially successful.     

Prevalence of vulval lichen planus in a cohort of women with oral lichen planus: an interdisciplinary study

BACKGROUND: Lichen planus (LP) is a mucocutaneous inflammatory dermatosis that frequently involves the oral and genital mucosae. Patients with LP affecting these sites are often seen by oral medicine specialists or gynaecologists who work in isolation and depend heavily on histopathologists to help them in confirming the diagnosis. There are few studies in the literature combining the experiences of these specialists who share the care of patients with both oral and genital LP. OBJECTIVES: To estimate the prevalence of vulval LP (VLP) in a cohort of patients with histologically confirmed oral LP (OLP). METHODS: The study group consisted of 42 women histologically diagnosed with OLP. The mean age was 60.5 years (range 27-81). They underwent genital examination, colposcopy and vulvoscopy. For the histological confirmation of clinical VLP biopsies were performed whenever a clinical lesion was found. Oral and genital biopsy specimens were processed through histological and immunohistochemical staining. Histological diagnoses of LP were made according to the modified World Health Organization histopathological criteria proposed by van der Meij and van der Waal for the diagnosis of OLP, and extended to VLP. Patients with clinical evidence, but without the histological confirmation of OLP and VLP, were excluded from the study group. RESULTS: Thirty-two vulval and one vaginal biopsy specimens were obtained. Histological diagnoses were confirmed in 24 of 32 (75%) patients who underwent a vulval biopsy: these represent 57% (24 of 42) of the study group. Of the 12 patients free of symptoms such as itching, burning and dyspareunia, but with clinical vulval lesions, 11 (92%) had histological confirmation of VLP. Vulval lichen sclerosus was ascertained in five of 32 (16%) cases. CONCLUSIONS: This study showed a 57% prevalence of VLP in selected patients with OLP. The high prevalence of VLP of 92% in the women who were free of vulval symptoms confirmed the usefulness of this careful integrated approach.     

Segmental lichen aureus: a report of two cases treated with methylprednisolone aceponate

Two cases of segmental lichen aureus with a response to topical 0.1% methylprednisolone aceponate ointment are reported. A 9-year-old child and a 23-year-old man showed complete resolution of their lesions following treatment with the latter after 7 months and 4 months, respectively. Lichen aureus is a rare form of the pigmented purpuric dermatoses characterized by golden-brown and lichenoid macules and papules, most often on the lower extremities. Segmental presentations have seldom been described. Histology showed a lichenoid infiltrate with extravasation of red blood cells and haemosiderin deposition. The aetiology is unclear and treatment is disappointing. We report an uncommon segmental presentation of lichen aureus with resolution of the lesions after treatment with a topical corticosteroid.     

Unexpected diminished innervation of epidermis and dermoepidermal junction in lichen amyloidosus

Background  Lichen amyloidosus is a localized, chronic, pruritic skin disease characterized by deposition of amyloid in the papillary dermis. The pathogenesis of the pruritus of lichen amyloidosus is largely unknown.
Objectives  To determine any change in the nerve fibre density in lichen amyloidosus lesions as an explanation for itch.
Methods  Using an antibody to protein gene product (PGP) 9.5, the immunohistochemical analysis of the skin biopsies of 30 Hispanic patients with clinicopathologically proven lichen amyloidosus and of 11 healthy Hispanic controls matched for age, sex and site was performed.
Results  Unexpectedly, the mean amount of PGP9.5 stain, a measure for nerve fibre amount, for the healthy controls was higher than the lichen amyloidosus group both in the epidermis ( P  <   0·0019) and dermoepidermal junction ( P  <   0·0064). No change was observed in the papillary dermis. Furthermore, the proportion of area covered by PGP9.5 showed a significant decrease in the epidermis ( P  <   0·0024) and dermoepidermal junction ( P  <   0·0075) in lichen amyloidosus compared with healthy controls. Age, gender and body site were found not to be influencing factors in nerve fibre amounts in lichen amyloidosus samples.
Conclusions  We speculate that the severe pruritus observed in lichen amyloidosus might be the result of the hypersensitivity of the remaining nerve fibres as a response to an unexplained neurodegeneration of the absent nerve fibres.     

Increased frequency of HLA-DR6 allele in Italian patients with hepatitis C virus-associated oral lichen planus

BACKGROUND: Recent controlled studies have confirmed that hepatitis C virus (HCV) is the main correlate of liver disease in patients with lichen planus (LP), mainly in southern Europe and Japan. However, a low prevalence of HCV infection has been found in LP patients in England and northern France, and significant differences in serum HCV RNA levels or HCV genotypes have not been found between LP patients and controls. Thus host rather than viral factors may be prevalent in the pathogenesis of HCV-related LP. The HLA-DR allele may influence both the outcome of HCV infection and the appearance of symptoms outside the liver. OBJECTIVES: To assess whether major histocompatibility complex class II alleles play a part in the development of HCV-related LP. METHODS: Intermediate-resolution DRB typing by hybridization with oligonucleotide probes was performed in 44 consecutive Italian oral LP (OLP) patients with HCV infection (anti-HCV and HCV RNA positive), in an age, sex and clinically comparable disease control group of 60 Italian OLP patients without HCV infection (anti-HCV and HCV RNA negative), and in 145 healthy unrelated Italian bone marrow donors without evidence of liver disease or history of LP and with negative tests for HCV. RESULTS: Patients with exclusive OLP and HCV infection possessed the HLA-DR6 allele more frequently than patients with exclusive OLP but without HCV infection (52% vs. 18%, respectively; Pc (Pcorrected) = 0.028, relative risk = 4.93). We did not find any relationship between mucocutaneous LP, HCV infection and HLA-DR alleles. CONCLUSIONS: HCV-related OLP therefore appears to be a distinctive subset particularly associated with the HLA class II allele HLA-DR6. This could partially explain the peculiar geographical heterogeneity of the association between HCV and LP.     

Impairment of myocardial functions and arterial stiffness in patients with lichen planus

BackgroundLichen planus is a chronic inflammatory mucocutaneous disease. Recent studies have suggested that it is associated with an increased risk of cardiovascular comorbidities.ObjectiveThe purpose of this study was to assess and compare arterial stiffness and cardiovascular hemodynamics in patients with lichen planus and a healthy control group.MethodsFifty-five patients with lichen planus and 42 healthy controls were enrolled. All patients underwent echocardiographic examination, and arterial stiffness was measured using applanation tonometry.ResultsNo statistically significant difference was determined between the patient and control groups in terms of arterial stiffness, but stiffness was markedly higher in patients with erosive lichen planus compared to the control group and other patients (p = 0.006, and p = 0.023, respectively). Moderate positive correlation was determined between duration of disease and arterial stiffness. Impairment of systolic and diastolic functions was also determined in patients with lichen planus compared to the control group (p < 0.001, and p = 0.005, respectively).Study limitationsRelatively low number of patients.ConclusionThe positive correlation observed between duration of disease and arterial stiffness in patients with lichen planus suggests that these patients should be followed-up in terms of cardiovascular risk in the presence of resistant and long-term disease, particularly in case of erosive lichen planus.     

Quantitative analysis of epithelial papillae in patients with oral lichen planus

Background  The oral mucosa is relatively vulnerable to pathological processes, and is often affected by autoimmune and malignant diseases. The oral epithelium is normally non-homogeneous, and joins to the connective tissue through interlocking of its downward projections in the form of papillae.
Objective  This study aims to conduct a histomorphometric study of the epithelial papillae in patients with oral lichen planus (OLP).
Material and method  This study was based on 100 cheek mucosa biopsies from patients with OLP (66 white reticular and 34 atrophic-erosive) (13 males and 87 females, with a mean age of 54.95 ± 13.64 years). A histological and morphometric evaluation was made, based on imaging analysis with MIP software 4.5 for studying the papillary structure in the patients with OLP.
Results  The mean epithelial thickness was 227.5 ± 78.5 µm. The different papillary measures – BLS (distance from basal layer to epithelial surface), DPS (distance from dermal papilla top to epithelial surface), DPW (dermal papilla width), and DPD (interdermal papilla distance between two papillae) – yielded no statistically significant differences with respect to age, sex, smoking and clinical form. However, a significant correlation was observed in relation to papilla width and inflammatory infiltrate ( P  = 0.031).
Conclusions  The application of this imaging system is useful for measuring variations in epithelial papillary architecture.

Conflicts of interest

None declared.     

microRNA-125b及其相关因子Bcl-2、 MMP-2在扁平苔藓中的表达

 目的:检测microRNA-125b（miRNA-125b）及其下游靶蛋白B细胞淋巴瘤-2 (Bcl-2) 蛋白、基质金属蛋白酶-2（MMP-2）在扁平苔藓(LP）中的表达,探讨其在发病中的意义。方法：采用RT-PCR法检测miRNA-125b在LP组织及正常皮肤组织的表达水平,ELISA法检测Bcl-2、MMP-2在LP组织及正常皮肤组织的表达水平,并进行比较。使用Spearman秩相关检验评估miRNA-125b与Bcl-2表达水平之间的相关性。结果：与正常组相比,LP患者组织中Bcl-2表达明显升高(0.52±0.05比0.49±0.04,t=2.84,P=0.018),MMP-2表达明显升高(7.48±2.88比5.78±3.82,t=2.19,P=0.032),而miRNA-125b表达明显下调(0.54±0.62比0.93±0.93,t=2.18,P=0.033)。Spearman秩相关分析显示miRNA-125b表达与Bcl-2呈负相关(r=-0.26,P=0.027),但与MMP-2无明显相关（r=-0.22,P=0.056）。结论：miRNA-125b可能是LP治疗的潜在治疗靶点,在LP发病机制中发挥关键作用。     

Cytokine alterations in lichen sclerosus: an immunohistochemical study

BACKGROUND: Although the histology of lichen sclerosus is characteristic, the precise nature of the inflammatory changes and the signals provoking them is uncertain. OBJECTIVES: To delineate the inflammatory changes in lichen sclerosus more accurately by studying cytokine changes. METHODS: An immunohistochemical study of 12 specimens of genital lichen sclerosus and one specimen of extragenital lichen sclerosus was undertaken using monoclonal antibodies to interferon (IFN)-gamma, IFN-gamma receptor, tumour necrosis factor (TNF)-alpha, interleukin (IL)-1alpha, IL-2 receptor (CD25), intercellular adhesion molecule-1 (ICAM-1) and its ligand CD11a. Control specimens were seven specimens of normal vulva obtained during gynaecological procedures, three specimens of normal skin, adjacent uninvolved thigh from three of the patients with lichen sclerosus, five specimens of nonvulval psoriasis, four specimens of nonvulval lichen planus and two specimens from chronic wounds. RESULTS: The lichen sclerosus specimens demonstrated slightly increased staining for IFN-gamma within the epidermis compared with the normal vulva and nonvulval skin. There was increased dermal staining for IFN-gamma both within the pale zone of the upper dermis and within the inflammatory zone below this. We confirmed our previous demonstration that in lichen sclerosus HLA-DR immunostaining is increased in association with vascular endothelium, the inflammatory cell infiltrate and around the keratinocytes. The areas of the epidermis with the strongest immunostaining for HLA-DR generally also had the strongest staining for IFN-gamma. In the lichen sclerosus specimens the zone of inflammation also demonstrated increased immunostaining for TNF-alpha, IL-1alpha, IFN-gamma receptor, CD25, CD11a and ICAM-1 while the zone of sclerosus demonstrated a smaller increase in immunostaining for IFN-gamma receptor, TNF-alpha, CD11a and ICAM-1, and the epidermis demonstrated increased staining for ICAM-1. CONCLUSIONS: The increased staining for IFN-gamma, TNF-alpha, IL-1alpha, IFN-gamma receptor, CD25, CD11a and ICAM-1 suggest that the cytokine response in lichen sclerosus shares characteristics of the cytokine response in lichen planus and chronic wounds.     

High incidence of lichen sclerosus in patients with squamous cell carcinoma of the penis

BACKGROUND: There is a well-documented association between lichen sclerosus (LS) and vulval carcinoma in women; however, until recently, there have only been anecdotal reports of penile squamous cell carcinoma (SCC) occurring in men with LS. OBJECTIVE: The incidence of penile carcinoma occurring on a background of LS remains uncertain, and we wished to examine this possible association further. METHOD: To address this, all the cases (n = 20) of penile SCC held on our pathology database (4 years) were examined. Histology was reviewed, blind to the clinical picture, for evidence of LS, applying strict histological criteria. Subsequently, clinical notes were reviewed for history of LS before the SCC presented, and history of previous circumcision, treatments, node involvement, metastases and death. RESULTS: In eight cases, evidence of LS was found in the excision specimen. Seven of these had well-differentiated SCC. In the 12 cases with no evidence of LS, only three were well differentiated. With case note review, seven had a history of LS (four with histological LS), sometimes preceding the SCC by 10 years. These all had well-differentiated SCC. Ten of the 20 patients are dead, seven from metastatic disease. Four deaths occurred in the 'well-differentiated LS' group, but only one from penile SCC metastatic disease. CONCLUSIONS: There appears to be a definite association between SCC of the penis and the presence of LS, similar to that reported between LS and vulval SCC in women. Of the 20 patients with penile SCC studied, 11 had a clinical history and/or histological evidence of LS. However, clinical presentation of the LS or need for circumcision may precede the SCC by many years. As follow-up is impractical, counselling at the time of diagnosis is very important, and it is essential that medical practitioners are aware of this association so that the subsequent risk from SCC is reduced.     

Psychological health in patients with genital and oral erosive lichen planus

BACKGROUND: Erosive lichen planus is a severe, recurrent and recalcitrant disease that affects several mucosal areas, mostly the genital area and the mouth, but also, for example, the oesophagus and perianal area. The disease causes serious symptoms, because of the raw, de-epithelialized mucosa and healing with scars/adhesions, which affect the patient's life in many ways. It causes, for example, difficulties in eating, drinking and going to the bathroom. Treatment is complicated and, so far, few therapeutic drugs other than steroids have been reported. OBJECTIVES: As the disease has severe implications on the patient's life it is important to investigate the psychological health of the patients, as well as the influence of stress on their health and wellbeing, in order to improve treatment. STUDY DESIGN, SUBJECTS AND METHODS: Forty-nine consecutive patients with erosive lichen planus were included during a 1-year period. The study was carried out as 'state-of-the-last-month', and stress, state anxiety, depression and 'erosive lichen planus factors', i.e. symptoms affecting daily life, were assessed. RESULTS: Eighty-seven per cent of the patients had symptoms, severely affecting daily life. Unexpectedly, oral symptoms seemed to be the most prominent. Our results showed that depression, anxiety and stress were more common in patients with erosive lichen planus than in a control group. DISCUSSION AND CONCLUSIONS: Erosive lichen planus is a severe disease with symptoms and complications affecting the patient's life. Our results indicate that their psychological health is also affected and emphasize the need for close collaboration between physicians, dentists with special knowledge in oral medicine and counsellors/psychologists to optimize handling of these patients.