线状盘状红斑狼疮伴皮肤钙沉着症1例

报告1例线状盘状红斑狼疮伴皮肤钙沉着症。患者女,36岁。因左侧头皮,额部,颧部和颊部带状萎缩性红斑20余年,局部起白色小结节2年就诊。皮肤科检查见左侧头皮额部,颧部和颊部有一纵行的带状萎缩性红斑,表面和毛细血管扩张和粘着性鳞屑,在额部萎缩性红斑上可见一半粒绿豆大的白色小结节,质硬,实验室检查示血钙和血磷均正常。分别取萎缩性红斑和白色小结节行组织病理检查,符合盘状红斑狼疮和皮肤钙沉着症组织病理学改变。     

Review of treatment for discoid lupus erythematosus

Discoid lupus erythematosus (DLE) is a chronic cutaneous disease characterized by inflammatory plaques that, in the absence of prompt diagnosis and treatment, may lead to disfiguring scarring and skin atrophy. However, there is limited evidence for which treatments are most effective. Currently, no medications have been approved specifically for the treatment of DLE. Many of the drugs described in the literature were developed for use in other immune disorders. This review will summarize current therapeutic options for DLE and their supporting evidence with discussion of prevention, topical measures, physical modalities, and systemic therapies, including newer potential therapies.     

Topical niacinamide (Nicotinamide) treatment for discoid lupus erythematosus (DLE): A prospective pilot study

Background

Cutaneous lupus erythematosus is an umbrella term for a group of autoimmune connective tissue disorders affecting the skin. Discoid lupus erythematosus (DLE) is the chronic condition and most common form of cutaneous lupus erythematosus.

Aims

Current therapies of DLE are challenging and not completely satisfactory, highly expensive, off-label, or poorly available (like antimalarials due to COVID-19 outbreaks). Nicotinamide, also called niacinamide, is a water-soluble form of vitamin B3 (niacin). Its multiple effects let us think that nicotinamide could be a therapy for lupus-associated skin lesions.

Methods

We performed a prospective randomized double-blind clinical trial on 60 subjects diagnosed with Discoid lupus erythematosus using topical Nicotinamide 2% and 4% preparations in form of cream and gel on skin and scalp lesions. Control group was included using only cream/gel base as placebo control.

Results

Obtained data showed that topical Nicotinamide can be used for the treatment of DLE as adjuvant to other treatment regimens with good cosmetic results and minimal side effects. Topical 4% Nicotinamide is superior to 2% preparation in response but associated with a higher incidence of irritation.

Conclusion

Topical Nicotinamide can be used for the treatment of DLE as an adjuvant to other treatment regimens with good cosmetic results and minimal side effects. Further trials with long-term therapy, follow-up period, and bigger sample sizes are required.     

Anti‐annexin 1 antibodies: A new diagnostic marker in the serum of patients with discoid lupus erythematosus

Abstract: Annexin 1 is an anti‐inflammatory molecule and has also been described to be a common target of autoantibodies. In this study, we determined whether antibodies against annexin 1 can be detected in sera of patients with cutaneous lupus erythematosus (CLE). Levels of anti‐annexin 1 antibodies were evaluated by a new established enzyme‐linked immunosorbent assay and found to be significantly higher in sera of patients with CLE when compared to normal healthy donors (NHD). Moreover, the percentage of sera positively tested for anti‐annexin 1 antibodies was elevated in patients with CLE when compared to NHD. In particular, the percentage of positive sera for anti‐annexin 1 antibodies was significantly higher in patients with discoid lupus erythematosus (DLE); however, disease activity did not correlate with the antibody levels. The results of this study indicate that anti‐annexin 1 antibodies in sera of patients with DLE might be a valuable aid in the diagnosis of this subtype.     

Treatment of chronic discoid lupus erythematosus with thalidomide

Summary In an uncontrolled study the therapeutic effect of thalidomide on chronic discoid lupus erythematosus was investigated. In 24 patients (17 women, 7 men) with a history ranging from 0.5–32 years and unresponsive to any previous therapeutic measures, complete or substantial regression of the disease was observed in 19 cases (80%) after treatment with thalidomide. Side effects were somnolence, constipation, exanthema, oral dryness, and circulatory disturbances. None of these effects were serious.     

Eyelid discoid lupus erythematosus and contact dermatitis: a case report

We report a patient with discoid lupus erythematosus (DLE) and associated allergic contact dermatitis (ACD) in the eyelids. In women, ACD caused by nail varnish is frequent and often seen in the eyelids. ACD caused by drugs (e.g. neomycin) is also frequent in this region. However, DLE with periorbital presentation without evidence of systemic or other cutaneous involvement is rare.     

The circulating lymphocyte profiles in patients with discoid lupus erythematosus and systemic lupus erythematosus suggest a pathogenetic relationship

BACKGROUND: Discoid lupus erythematosus (DLE) and systemic lupus erythematosus (SLE) are chronic inflammatory diseases of unknown aetiology; the relationship of DLE with SLE has been a subject of debate for many years. OBJECTIVES; To find evidence for systemic immune activation in DLE by analysis of the immunophenotypic profiles of circulating lymphocytes, and to compare these changes with those in patients with SLE. METHODS: The immunophenotypic profile of peripheral blood lymphocyte subsets from 23 DLE patients without clinical or laboratory evidence of systemic disease, 25 SLE patients and 38 healthy donors was characterized by two-colour immunofluorescence flow cytometry analysis. None of the patients was receiving corticosteroid or immunosuppressive treatment. RESULTS: Patients with DLE had increased numbers of circulating HLA-DR+ CD3+ T cells and HLA-DR+ CD4+ T cells, indicating systemic T-cell activation, and an expansion of CD5+ CD19+ B cells. Decreased numbers of T-cell subsets expressing the differentiation markers CD11b and CD16/56, and of CD16/56+ natural killer cells were also found. In SLE, the changes were similar but more pronounced. In addition, a profound CD4+ T-cell lymphopenia and an increase of HLA-DR+ CD8+ T cells were found only in SLE. CONCLUSIONS: Our data provide evidence for systemic activation of the cellular immune system in patients with purely cutaneous DLE. Similarities in the lymphocyte immunophenotypic profiles in patients with DLE compared with SLE suggest that there are common immunopathological processes in these two conditions.     

Comparative analysis of subacute cutaneous lupus erythematosus and chronic cutaneous lupus erythematosus: clinical and immunological study of 270 patients

Background Lupus erythematosus (LE) is a chronic, autoimmune disease resulting from an interaction of genetic, environmental and hormonal factors and characterized by a spectrum of clinical forms with variable evolution from a localized cutaneous form to a life‐threatening systemic form. Objective To analyse and compare the prevalence and characteristics of the main clinical and immunological manifestations of subacute cutaneous LE (SCLE) and chronic CLE (CCLE). Methods A total of 270 patients with CLE (112 patients with SCLE and 158 patients with CCLE) were studied retrospectively. The clinical and serological characteristics of all the patients were collected in a chart review. Results The patients with SCLE had a higher prevalence of annular and papulosquamous lesions, Raynaud phenomenon, mucous membrane ulcers, malar rashes, photosensitivity, vasculitis and a lower frequency of discoid lesions and alopecia compared with patients with CCLE. Patients with SCLE had a higher prevalence of arthralgias (P < 0·001), xerophthalmia (P = 0·045), arthritis (P < 0·001), nephropathy (P = 0·003) and systemic LE (SLE) (P < 0·001) compared with patients with CCLE. Patients with SCLE also had a higher frequency of laboratory and serological abnormalities than patients with CCLE. Generalized discoid LE (DLE) was associated with a higher prevalence of photosensitivity (P < 0·001), panniculitis (P = 0·009) and SLE (P = 0·003) than localized DLE. In patients with SCLE and those with CCLE, photosensitivity, arthralgias, arthritis, nephropathy and xerophthalmia were associated with SLE. In patients with SCLE, significant correlations existed between clinical and immunological data. Conclusions In our series, differences in the expression of CCLE and SCLE were found with respect to the distribution and type of lesions, systemic features and immunological findings.     

Photoprotection by thalidomide in patients with chronic cutaneous and systemic lupus erythematosus: discordant effects on minimal erythema dose and sunburn cell formation

BACKGROUND: Thalidomide is an anti-inflammatory and immunomodulatory agent with proven efficacy in several refractory inflammatory skin conditions including photoexacerbated skin diseases. The effects of thalidomide on ultraviolet (UV)-induced cutaneous damage in humans have not been extensively studied. We describe the results of minimal erythema dose (MED) testing in nonlesional skin of three patients with chronic cutaneous lupus erythematosus (CCLE) before and after treatment with thalidomide. OBJECTIVES: To determine whether thalidomide treatment provides clinical and histological evidence of photoprotection from acute UV injury. METHODS: MED testing was performed in nonlesional skin of three patients with CCLE before and after treatment with thalidomide. Skin biopsy specimens were taken from MED sites for in situ immunochemistry. RESULTS: In each patient, the MED to UVB irradiation was significantly higher while the patient was receiving thalidomide treatment than in the absence of thalidomide, suggesting a systemic photoprotective effect. Thalidomide treatment had no significant effect on markers of apoptosis including sunburn cell formation and terminal deoxynucleotidyl transferase-mediated biotinylated deoxyuridine triphosphate nick end labelling, which identifies single-strand breaks in DNA. CONCLUSIONS: Thalidomide inhibits acute UVB erythema at 24 h after exposure, as a 100-mg daily dose of this drug for 4 weeks conveyed a sun protection factor of 1.56 to > 4.0. We conclude that inhibition of UVB-induced inflammation may, in part, explain the therapeutic benefits of this agent on photosensitive diseases.     

Squamous cell carcinoma complicating chronic discoid lupus erythematosus (CDLE)

Occurrence of squamous cell carcinoma with some unusual clinical features is described in two female patients.     

Cutaneous lupus erythematosus: a personal approach to management

SUMMARY Skin disease in patients with lupus erythematosus may be subdivided into two broad categories - those lesions that when biopsied demonstrate interface dermatitis and those that do not demonstrate interface dermatitis. The skin lesions that are represented by the interface dermatitis include discoid lupus erythematosus, subacute cutaneous lupus erythematosus and acute cutaneous lupus erythematosus. Patients with these 'specific' manifestations have varying degrees of systemic involvement from rare systemic disease in patients with localized discoid lupus erythematosus to common and often severe involvement in patients with acute cutaneous lupus erythematosus. Patients who do not demonstrate interface dermatitis also may have systemic disease and in some instances the skin manifestations are linked to some of the more severe systemic manifestations. Many patients with cutaneous lesions characterized by the interface dermatitis can be controlled with 'standard' therapies including sunscreens, protective clothing and behavioural alteration, and topical corticosteroids with or without an oral antimalarial agent. This review presents a brief summary of each common cutaneous manifestation of lupus erythematosus, its relationship to systemic involvement and treatment issues to effectively deal with the lupus erythematosus patient who has skin disease.     

Epidemiology of cutaneous lupus erythematosus in a tertiary referral centre in Singapore

The aim of this retrospective study was to investigate the epidemiology, yield of investigations and proportion of patients who develop systemic lupus erythematosus (SLE) among the subsets of cutaneous lupus erythematosus (LE) in the Singapore Asian population. One hundred and twenty-five patients were diagnosed with cutaneous LE on clinico-pathological correlation, of which 73 had discoid lupus erythematosus (DLE), eight had subacute cutaneous LE (SCLE), 22 had acute LE lesions and the remainder had other less common forms of cutaneous LE. Histology was consistent with LE in 94.4% and suggestive in 4.8%. Direct immunofluorescence was positive in 61% of DLE, 86% of SCLE and 80% of acute LE cases. Antinuclear antibody (ANA) was present in the majority of acute LE (85%) and SCLE (88%) but only in 25% of DLE. Eight patients (11%) presenting with DLE had definite SLE at first presentation and two (2.7%) subsequently several months later. Of these patients, six had only mucocutaneous and serological criteria but two had major organ involvement. Five SCLE patients (63%) fulfilled the criteria for SLE, including two with major organ involvement.     

Suppressor activity of peripheral blood mononuclear cells in patients with chronic discoid lupus erythematosus

Summary Significantly increased suppressor activity of peripheral blood mononuclear cells was demonstrated in patients suffering from chronic discoid lupus erythematosus.This work was supported by the Scientific Research Council, Ministry of Health, Hungary (Code No. 421 030401 1/S)     

疣状盘状红斑狼疮1例

患者女，６２岁，病期２６年。躯干上部、面及上肢疣状盘状斑块、刺状角栓，组织病理象为表皮呈疣状增生及胶样小体。病程慢性，无特效疗法。     

Successful treatment of facial localized discoid lupus erythematosus with intralesional betamethasone: A report of three cases

Discoid lupus erythematosus (DLE) is a chronic autoimmune skin disease that usually causes disfiguring scarring, dyspigmentation, and atrophy. Despite a range of available topical and systemic therapies, the treatment of DLE remains a therapeutic challenge, especially in some refractory cases. Here, we reported three male patients with long‐term chronic lesions of unilateral facial localized DLE, who failed to have their disease controlled with many previous topical/systemic treatments, showed rapid and well response to intralesional injections of betamethasone (2 mg/mL, 0.2 mL/site) monotherapy once every 2 weeks for two, two, and four times of treatment, respectively. Intralesional betamethasone may provide a safe and effective alternative in the management of refractory localized DLE skin lesions.     

Measuring disease activity and damage in discoid lupus erythematosus

Background Discoid lupus erythematosus (DLE) is a disfiguring inflammatory skin disease. There is no specific tool for measuring disease severity. Objectives To determine the features needed in a score measuring activity and damage in DLE and to investigate the score’s reliability and its correlation with the physician’s global assessment of disease severity and the patient‐reported Dermatology Quality of Life Index (DLQI). Methods The content of the score was determined following a peer review, pilot work in patients and a preliminary inter‐rater reliability study. The Score of Activity and Damage in DLE (SADDLE) measures severity of activity (erythema, scale, induration) and damage (scarring/atrophy and dyspigmentation) attributable to DLE. Summed scores range between 0 and 195. Inter‐ and intrarater reliability of the score was tested using six assessors and nine patients with DLE. Intraclass correlation coefficients (ICCs) > 0·7 were considered evidence of good inter‐ and intrarater agreement. Results The mean ± SD SADDLE score of nine patients in the inter‐rater reliability study was 47 ± 22 (range 14–102). There was good inter‐rater agreement for the total score [ICC 0·82; 95% confidence interval (CI) 0·61–0·95] and for the activity and damage scales, the individual physical signs and the total scores at individual body sites. The total score demonstrated excellent intrarater reliability (ICC 0·98; 95% CI 0·86–1·00). Although there was poor inter‐rater agreement for global assessments (ICC 0·28; 95% CI 0·06–0·66), a good correlation was demonstrated between total scores and global assessments (r = 0·7). A weaker positive correlation was observed between disease activity scores and DLQI (r = 0·4). Conclusions The SADDLE measures activity and damage in patients with DLE. It demonstrates good inter‐ and excellent intrarater agreement, over and above that for global assessment. It correlates well with global assessment scores. Further studies are required to investigate SADDLE’s responsiveness to change with therapy.