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

A large amount of knowledge has been acquired since the original descriptions of Lyme borreliosis (LB) and of its causative agent, Borrelia burgdorferi sensu stricto. The complexity of the organism and the variations in the clinical manifestations of LB caused by the different B. burgdorferi sensu lato species were not then anticipated. Considerable improvement has been achieved in detection of B. burgdorferi sensu lato by culture, particularly of blood specimens during early stages of disease. Culturing plasma and increasing the volume of material cultured have accomplished this. Further improvements might be obtained if molecular methods are used for detection of growth in culture and if culture methods are automated. Unfortunately, culture is insensitive in extracutaneous manifestations of LB. PCR and culture have high sensitivity on skin samples of patients with EM whose diagnosis is based mostly on clinical recognition of the lesion. PCR on material obtained from extracutaneous sites is in general of low sensitivity, with the exception of synovial fluid. PCR on synovial fluid has shown a sensitivity of up to >90% (when using four different primer sets) in patients with untreated or partially treated Lyme arthritis, making it a helpful confirmatory test in these patients. Currently, the best use of PCR is for confirmation of the clinical diagnosis of suspected Lyme arthritis in patients who are IgG immunoblot positive. PCR should not be used as the sole laboratory modality to support a clinical diagnosis of extracutaneous LB. PCR positivity in seronegative patients suspected of having late manifestations of LB most likely represents a false-positive result. Because of difficulties in direct methods of detection, laboratory tests currently in use are mainly those detecting antibodies to B. burgdorferi sensu lato. Tests used to detect antibodies to B. burgdorferi sensu lato have evolved from the initial formats as more knowledge on the immunodominant antigens has been collected. The recommendation for two-tier testing was an attempt to standardize testing and improve specificity in the United States. First-tier assays using whole-cell sonicates of B. burgdorferi sensu lato need to be standardized in terms of antigen composition and detection threshold of specific immunoglobulin classes. The search for improved serologic tests has stimulated the development of recombinant protein antigens and the synthesis of specific peptides from immunodominant antigens. The use of these materials alone or in combination as the source of antigen in a single-tier immunoassay may someday replace the currently recommended two-tier testing strategy. Evaluation of these assays is currently being done, and there is evidence that certain of these antigens may be broadly cross-reactive with the B. burgdorferi sensu lato species causing LB in Europe.

Diagnosis of Lyme Borreliosis

https://jcp.bmj.com/content/jclinpath/33/9/907.1.full.pdf

Principles and Practice of Infectious Diseases

Eight of the more than 80 known herpesviruses are human pathogens. Human herpes simplex virus (HSV) is a contagious infection with a large reservoir in the general population. It has a potential for significant complications in the immunocompromised host. In addition, psychological distress caused by the negative stigma associated with genital herpes and visible facial lesions in those experiencing frequent outbreaks renders it a challenging clinical dilemma. This article reviews the epidemiology, pathogenesis, and diagnostic features of HSV infections, providing the clinician with an up-to-date understanding of the available management strategies for mucocutaneous HSV-induced disease.

Human herpes simplex virus infections: epidemiology, pathogenesis, symptomatology, diagnosis, and management.

Syphilis is a chronic sexually transmitted disease caused by Treponema pallidum subsp. pallidum. Clinical manifestations separate the disease into stages; late stages of disease are now uncommon compared to the preantibiotic era. T. pallidum has an unusually small genome and lacks genes that encode many metabolic functions and classical virulence factors. The organism is extremely sensitive to environmental conditions and has not been continuously cultivated in vitro. Nonetheless, T. pallidum is highly infectious and survives for decades in the untreated host. Early syphilis lesions result from the host's immune response to the treponemes. Bacterial clearance and resolution of early lesions results from a delayed hypersensitivity response, although some organisms escape to cause persistent infection. One factor contributing to T. pallidum's chronicity is the paucity of integral outer membrane proteins, rendering intact organisms virtually invisible to the immune system. Antigenic variation of TprK, a putative surface-exposed protein, is likely to contribute to immune evasion. T. pallidum remains exquisitely sensitive to penicillin, but macrolide resistance has recently been identified in a number of geographic regions. The development of a syphilis vaccine, thus far elusive, would have a significant positive impact on global health.

/pdf/biological-basis-for-syphilis-57y72ibofo.pdf

Biological Basis for Syphilis

To the Editor: Patients with erythropoietic protoporphyria, a rare inherited disease, accumulate photosensitizing protoporphyrin in the dermis, which results in severe dermal pain and incapacitating phototoxic reactions when the skin is exposed to visible light, primarily blue light (the Soret band, with the strongest absorbance among the porphyrins).1 Current treatments are partially effective at best (unpublished data). A beneficial effect of melanogenesis induced by natural sunlight or by ultraviolet radiation has been described in anecdotal reports.2,3 Afamelanotide (Nle4-D-Phe7–α-melanocyte–stimulating hormone, formerly called CUV1647) is an α-melanocyte–stimulating hormone analogue that induces epidermal melanin formation.4 Here, we describe the responses . . .

An alpha-melanocyte-stimulating hormone analogue in erythropoietic protoporphyria.

Background: Eosinophilic fasciitis (EF) is a rare fibrosing disorder associated with peripheral eosinophilia and scleroderma-like induration of the distal extremities which affects substantially quality of life. Although the disease has been described 30 years ago, the etiology and pathomechanisms are still obscure, and consensus for therapy is lacking. Numerous case reports of patients with EF exist but series are scarce. Patients and Methods: Eleven patients with EF from the Department of Dermatology, Kantonsspital Aarau, the University Hospital Basel and the Outpatient Clinic of Dermatology, Triemli Hospital Zurich, Switzerland, were retrospectively studied. Results: In 4 patients the initial diagnosis was not recognized by the referring nondermatologists. The median age was 55 years, excluding the youngest patient ever diagnosed with EF (age = 1 year). All patients showed an induration of the skin, which led to painful contractures in the joints in 3 cases. All but 2 patients demonstrated edema. A slight predominance of the upper extremities was observed. Sclerodactyly was noticed in 1 patient. Three patients reported an initial trauma at the affected site. Two patients were tested positive for borreliosis. One patient subsequently developed aplastic anemia and Hashimoto thyroiditis. Visceral or extracutaneous involvement was absent. Eight patients had a full or partial recovery under corticosteroids whereas in 2, improvement could be achieved only with cyclosporine, azathioprine or cyclophosphamide. Conclusions: The diagnosis of EF can be established by clinical, laboratory and histological findings. In general, corticosteroids are highly efficacious in EF and only a minority of patients need other immunosuppressive or cytostatic drugs.

Eosinophilic fasciitis 30 years after - what do we really know? Report of 11 patients and review of the literature.

(See the Editorial Commentary by Hook, on pages 1623–4.)

With 12 million new cases a year worldwide, syphilis remains a global problem. According to the World Health Organization, >90% of cases occur in developing countries [1]. Rising incidence has been reported in many European countries since the mid-1990s [2]. Similarly in the United States, a resurgence of syphilis has been noted after a nadir in 2000 [3].

For laboratory diagnosis of syphilis and for monitoring treatment response, serological testing is the most important approach. In patients coinfected with human immunodeficiency virus (HIV), atypical serological courses have been reported. Although the clinically defined treatment response does not seem to be influenced by HIV coinfection, serologically defined treatment failure has been reported [4]. This might be a consequence of slower Venereal Disease Laboratory Research (VDRL) test seroreversion in HIV-infected individuals [5]. However, when reviewing previous studies in this field, no firm conclusion can be drawn regarding whether HIV coinfection significantly alters the treatment response. This issue still is important to address, as today most HIV-infected individuals are treated with highly active antiretroviral therapy and probably have a restored immune system. It is likely that this changed the response to syphilis treatment in HIV patients.

We aimed to compare the serological response to treatment between the different stages of syphilis and to examine the influence of HIV coinfection.

https://www.zora.uzh.ch/id/eprint/69253/1/HIV_Status.pdf

Serological Response to Treatment of Syphilis According to Disease Stage and HIV Status

Objective To systematically review and estimate pooled sensitivity and specificity of the polymerase chain reaction (PCR) technique compared to recommended reference tests in the diagnosis of suspected syphilis at various stages and in various biological materials. Design Systematic review and meta-analysis. Data sources Search of three electronic bibliographic databases from January 1990 to January 2012 and the abstract books of five congresses specialized in the infectious diseases' field (1999-2011). Search key terms included syphilis, Treponema pallidum or neurosyphilis and molecular amplification, polymerase chain reaction or PCR. Review methods We included studies that used both reference tests to diagnose syphilis plus PCR and we presented pooled estimates of PCR sensitivity, specificity, and positive and negative likelihood ratios (LR) per syphilis stages and biological materials. Results Of 1160 identified abstracts, 69 were selected and 46 studies used adequate reference tests to diagnose syphilis. Sensitivity was highest in the swabs from primary genital or anal chancres (78.4%; 95% CI: 68.2-86.0) and in blood from neonates with congenital syphilis (83.0%; 55.0-95.2). Most pooled specificities were ∼95%, except those in blood. A positive PCR is highly informative with a positive LR around 20 in ulcers or skin lesions. In the blood, the positive LR was <10. Conclusions The pooled values of LR showed that T. pallidum PCR was more efficient to confirm than to exclude syphilis diagnosis in lesions. PCR is a useful diagnostic tool in ulcers, especially when serology is still negative and in medical settings with a high prevalence of syphilis.

https://sti.bmj.com/content/sextrans/89/3/251.full.pdf

Sensitivity, specificity and likelihood ratios of PCR in the diagnosis of syphilis: a systematic review and meta-analysis

After a marked decline in the number of syphilis cases in the context of AIDS prevention campaigns, a significant increase has been observed in states of the former Soviet Union since 1994. In recent years, outbreaks have also been reported in the US, Canada, and several European countries. The current epidemic in the US and in different parts of Europe has largely involved men who have sex with men, many of whom are infected with HIV. Since a misdiagnosis of syphilis can have serious consequences for the patient and also for pregnancies and newborns, clinicians should be aware of the many manifestations of syphilis and difficulties in the diagnosis and management of the disease. Younger clinicians in particular are no longer familiar with the diverse clinical symptoms and the complex diagnostics of syphilis. Patients co-infected with HIV may present with atypical clinical manifestations and laboratory test results. Furthermore, through its association with an increased risk of HIV infection, syphilis has acquired a new potential for morbidity and mortality, and the diagnosis of syphilis should be routinely considered in patients with uveitis, sudden deafness, aortic thoracic aneurysm, or pregnancy. Only a minority of syphilis infections are detected in the primary stage. This may be because of atypical locations and, occasionally, atypical morphology of the lesions; however, it may also be because of the difficulty of detecting the pathogen. In the secondary stage, which is clinically extremely diverse, the diagnosis is confirmed serologically. There is a need for increased awareness of the symptoms and signs of acute infections, together with a willingness to consider the diagnosis of syphilis in patients with vague symptoms. An increasing number of diagnostic tests (both specific and nonspecific) are now available. However, in the absence of clinical symptoms or in cases with a low titer or inconsistent test results, diagnosis of syphilis can be difficult or even impossible. Treatment and follow-up should follow current guidelines designed for the involved area. In this article, the cutaneous manifestations of syphilis and their diagnostic and therapeutic management are described in detail.

Stephan Lautenschlager

Papers

An alpha-melanocyte-stimulating hormone analogue in erythropoietic protoporphyria.

Eosinophilic fasciitis 30 years after - what do we really know? Report of 11 patients and review of the literature.

Serological Response to Treatment of Syphilis According to Disease Stage and HIV Status

Sensitivity, specificity and likelihood ratios of PCR in the diagnosis of syphilis: a systematic review and meta-analysis

Cutaneous manifestations of syphilis : recognition and management.