结节病易误诊,据王洪武等~([1])收集国内18篇关于此病误诊的文献,误诊率高达63.2%,当然有误诊就会有误治,如孙永昌等~([2])报道26例结节病在影像学检查诊断的第一印象中拟诊结核5例,其中就有2例完成规范的抗结核治疗,为此应引起临床对本病诊治的重视。

Sarcoidosis

Our objective was to update the EULAR recommendations for the management of systemic lupus erythematosus (SLE), based on emerging new evidence. We performed a systematic literature review (01/2007-12/2017), followed by modified Delphi method, to form questions, elicit expert opinions and reach consensus. Treatment in SLE aims at remission or low disease activity and prevention of flares. Hydroxychloroquine is recommended in all patients with lupus, at a dose not exceeding 5 mg/kg real body weight. During chronic maintenance treatment, glucocorticoids (GC) should be minimised to less than 7.5 mg/day (prednisone equivalent) and, when possible, withdrawn. Appropriate initiation of immunomodulatory agents (methotrexate, azathioprine, mycophenolate) can expedite the tapering/discontinuation of GC. In persistently active or flaring extrarenal disease, add-on belimumab should be considered; rituximab (RTX) may be considered in organ-threatening, refractory disease. Updated specific recommendations are also provided for cutaneous, neuropsychiatric, haematological and renal disease. Patients with SLE should be assessed for their antiphospholipid antibody status, infectious and cardiovascular diseases risk profile and preventative strategies be tailored accordingly. The updated recommendations provide physicians and patients with updated consensus guidance on the management of SLE, combining evidence-base and expert-opinion.

https://ard.bmj.com/content/annrheumdis/78/6/736.full.pdf

2019 update of the EULAR recommendations for the management of systemic lupus erythematosus

Psoriasis is a common, chronic, inflammatory, multisystem disease with predominantly skin and joint manifestations affecting approximately 2% of the population. In this second of 5 sections of the guidelines of care for psoriasis, we give an overview of psoriatic arthritis including its cardinal clinical features, pathogenesis, prognosis, classification, assessment tools used to evaluate psoriatic arthritis, and the approach to treatment. Although patients with mild to moderate psoriatic arthritis may be treated with nonsteroidal anti-inflammatory drugs and/or intra-articular steroid injections, the use of disease-modifying antirheumatic drugs, particularly methotrexate, along with the biologic agents, are considered the standard of care in patients with more significant psoriatic arthritis. We will discuss the use of disease-modifying antirheumatic drugs and the biologic therapies in the treatment of patients with moderate to severe psoriatic arthritis.

https://www.jaad.org/article/S0190-9622(09)00012-7/pdf

Guidelines of care for the management of psoriasis and psoriatic arthritis: Section 2. Psoriatic arthritis: Overview and guidelines of care for treatment with an emphasis on the biologics

Research on psoriasis pathogenesis has largely increased knowledge on skin biology in general. In the past 15 years, breakthroughs in the understanding of the pathogenesis of psoriasis have been translated into targeted and highly effective therapies providing fundamental insights into the pathogenesis of chronic inflammatory diseases with a dominant IL-23/Th17 axis. This review discusses the mechanisms involved in the initiation and development of the disease, as well as the therapeutic options that have arisen from the dissection of the inflammatory psoriatic pathways. Our discussion begins by addressing the inflammatory pathways and key cell types initiating and perpetuating psoriatic inflammation. Next, we describe the role of genetics, associated epigenetic mechanisms, and the interaction of the skin flora in the pathophysiology of psoriasis. Finally, we include a comprehensive review of well-established widely available therapies and novel targeted drugs.

Psoriasis Pathogenesis and Treatment.

Of the 131 studies on monotherapy or combination therapy assessed, 56 studies on the different forms of phototherapy fulfilled the criteria for inclusion in the guidelines. Approximately three-quarters of all patients treated with phototherapy attained at least a PASI 75 response after 4 to 6 weeks, and clearance was frequently achieved (levels of evidence 2 and 3). Phototherapy represents a safe and very effective treatment option for moderate to severe forms of psoriasis vulgaris. The onset of clinical effects occurs within 2 weeks. Of the unwanted side effects, UV erythema from overexposure is by far the most common and is observed frequently. With repeated or long-term use, the consequences of high, cumulative UV doses (such as premature aging of the skin) must be taken into consideration. In addition, carcinogenic risk is associated with oral PUVA and is probable for local PUVA and UVB. The practicability of the therapy is limited by spatial, financial, human, and time constraints on the part of the physician, as well as by the amount of time required by the patient. From the perspective of the cost-bearing institution, phototherapy has a good cost-benefit ratio. However, the potentially significant costs for, and time required of, the patient must be considered.

https://www.huidziekten.nl/richtlijnen/european-guideline-psoriasis-2009.pdf

European S3-guidelines on the systemic treatment of psoriasis vulgaris

Background : Psoriasis vulgaris may benefit from treatment with fumaric acid and/or its derivatives; however, because different preparations have been used, results have been contradictory and difficult to interpret. Objective : The purpose of this clinical trial was to evaluate the therapeutic value of fumaric acid derivatives. Methods : A randomized double-blind study was carried out in patients with psoriasis, comparing a well-characterized formulation of fumaric acid derivatives with placebo. Results : The results indicated statistically significant superiority of the fumaric acid derivatives over placebo. Adverse events (flush, gastrointestinal disturbances) were initially relatively frequent, but decreased thereafter. Conclusion : Fumaric acid derivatives were found to be effective and safe in the treatment of psoriasis.

Antipsoriatic effect of fumaric acid derivatives. Results of a multicenter double-blind study in 100 patients.

Topical treatment of cutaneous lupus erythematosus usually includes potent glucocorticosteroids. However, prolonged use causes adverse side effects including skin atrophy as the foremost concern. In contrast to glucocorticosteroids, the anti-inflammatory and immunosuppressive macrolactam pimecrolimus has no atrophogenic potential. Affected areas of 11 patients with different forms of lupus erythematosus were treated with pimecrolimus 1% cream under semiocclusive conditions twice daily for 3 weeks. Skin involvement before and after therapy was assessed by means of a clinical score. In all patients, significant regression of skin lesions was observed after therapy (P <.001). This was an open and uncontrolled study on a limited number of cases. We suggest that pimecrolimus 1% cream could be an efficacious and safe treatment option for cutaneous lupus erythematosus.

https://www.jaad.org/article/S0190-9622(04)00521-3/pdf

Pimecrolimus 1% cream for cutaneous lupus erythematosus

Summary
Background Necrobiosis lipoidica (NL) is an uncommon granulomatous skin disease with association to diabetes mellitus. To date, no proven effective therapy for NL has been implemented. The standard treatment is topical application of corticosteroids, but numerous agents have been reported for NL, with varying degrees of success. In recent case reports, fumaric acid esters (FAE) have been reported to be effective in granulomatous skin diseases such as granuloma annulare, cutaneous sarcoidosis and NL.

Objectives  We sought to investigate the efficacy of FAE in a larger number of patients with NL.

Methods  Eighteen patients with histopathologically proven NL were consecutively recruited into a prospective noncontrolled study. Dosage of FAE was given according to the standard therapy regimen for psoriasis. FAE were administered for at least 6 months. The treatment outcome was evaluated by means of clinical and histological scoring and 20-MHz ultrasound assessments.

Results  Three patients discontinued therapy with FAE, while the remaining 15 patients finished the study. After a mean ± SD treatment period of 7·7 ± 2·9 months, a significant (P < 0·001) decrease in the mean ± SD clinical score, from 7·4 ± 1·8 at the beginning to 2·5 ± 1·3 at the end of therapy, was observed. Significant clinical improvement of NL was accompanied by significant (P = 0·019) increase of dermal density as assessed by means of 20-MHz ultrasound, and significant (P = 0·011) reduction of the histological score. Adverse effects were moderate and consisted mainly of gastrointestinal complaints and flushing. During follow-up of at least 6 months, clinical outcome remained stable in all patients.

Conclusions  The results of this study demonstrate that FAE are beneficial and safe in the treatment of patients with NL.

Fumaric acid esters in necrobiosis lipoidica: results of a prospective noncontrolled study.

Letters to Dermatology dominance of CD4+ T lymphocytes in the inflammatory infiltrate supports this notion [17, 18]. The importance of T cells in the pathogenesis of AEGCG is also underlined by a case of adult T-cell leukaemia accompanied by AEGCG [19]. The same mechanism of elastolysis may also be operative in granulomatous slack skin, a rare cutaneous elastolytic disorder that has turned out to be a special variant of CD4+ cutaneous T-cell lymphoma [20]. In contrast to this latter disease, elastolysis in AEGCG seems to be a temporal phenomenon as spontaneous complete remissions have been reported in some patients [1, 9]. Other differential diagnoses of AEGCG include necrobiosis lipoidica, which is histologically characterised by areas of necrobiosis, palisading granulomas, lipid depositions, vascular wall thickening and capillary endothelial proliferation [5]. Clinically, necrobiosis lipoidica shows a sclerotic reaction pattern rather than the atrophy seen in AEGCG. As for the management of AEGCG, no standard therapy is established, and spontaneous remissions may also occur [1, 9]. Successfully used treatments included excision of a solitary lesion [17], intralesional or systemic corticosteroids [1, 10], retinoid-PUVA [19], cyclosporine [18], tranilast [9] and dapsone [21]. Clofazimine was beneficial for 1 patient [17], but led to deterioration of the skin disease in another individual, who was then successfully treated with chloroquine [4]. In our patient, partial remission could be achieved with 410 mg chloroquine daily. Altogether, chloroquine treatment failed in 3 cases and showed a partial effect in another 3 cases [1]. A few patients were treated without success by cryotherapy [22], cauterisation [23], topical steroids [1, 2], PUVA [1, 19] and methotrexate [18]. The therapy of AEGCG remains unsatisfactory, and various treatment options have to be considered for patients with this rather rare disease.

Frank M. Pawlak

Papers

Antipsoriatic effect of fumaric acid derivatives. Results of a multicenter double-blind study in 100 patients.

Pimecrolimus 1% cream for cutaneous lupus erythematosus

Fumaric acid esters in necrobiosis lipoidica: results of a prospective noncontrolled study.

Clearance of Necrobiosis lipoidica with Fumaric Acid Esters