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

Although the rate of acute severe adverse cutaneous reactions to medications is low, these reactions can affect anyone who takes medications and can result in death or disability1. Even a small number of cases associated with a particular drug may alter the recommendations for its use2–4. Prompt differentiation of severe adverse cutaneous reactions from less serious skin disorders may be difficult. Rapid recognition of severe reactions is essential. Prompt withdrawal of the offending drug is often the most important action to minimize morbidity. Adverse cutaneous reactions to drugs are frequent, affecting 2 to 3 percent of hospitalized . . .

Severe Adverse Cutaneous Reactions to Drugs

Heparin and low-molecular-weight heparin: mechanisms of action, pharmacokinetics, dosing considerations, monitoring, efficacy, and safety.

This Review outlines the understanding and management of triple-negative breast cancer (TNBC). TNBC shares morphological and genetic abnormalities with basal-like breast cancer (BLBC), a subgroup of breast cancer defined by gene-expression profiling. However, TNBC and BLBC tumors are heterogeneous and overlap is incomplete. Breast cancers found in BRCA1 mutation carriers are also frequently triple negative and basal like. TNBC and BLBC occur most frequently in young women, especially African Americans, and tend to exhibit aggressive, metastatic behavior. These tumors respond to conventional chemotherapy but relapse more frequently than hormone receptor-positive, luminal subtypes and have a worse prognosis. New systemic therapies are urgently needed as most patients with TNBC and/or BLBC relapse with distant metastases, and hormonal therapies and HER2-targeted agents are ineffective in this group of tumors. Poly (ADP-ribose) polymerase inhibitors, angiogenesis inhibitors, EGFR-targeted agents, and src kinase and mTOR inhibitors are among the therapeutic agents being actively investigated in clinical trials in patients with TNBC and/or BRCA1-associated tumors. Increased understanding of the genetic abnormalities involved in the pathogenesis of TNBC, BLBC and BRCA1-associated tumors is opening up new therapeutic possibilities for these hard-to-treat breast cancers.

Triple-negative breast cancer: disease entity or title of convenience?

Background Inherited ichthyoses belong to a large, clinically and etiologically heterogeneous group of mendelian disorders of cornification, typically involving the entire integument. Over the recent years, much progress has been made defining their molecular causes. However, there is no internationally accepted classification and terminology. Objective We sought to establish a consensus for the nomenclature and classification of inherited ichthyoses. Methods The classification project started at the First World Conference on Ichthyosis in 2007. A large international network of expert clinicians, skin pathologists, and geneticists entertained an interactive dialogue over 2 years, eventually leading to the First Ichthyosis Consensus Conference held in Soreze, France, on January 23 and 24, 2009, where subcommittees on different issues proposed terminology that was debated until consensus was reached. Results It was agreed that currently the nosology should remain clinically based. "Syndromic" versus "nonsyndromic" forms provide a useful major subdivision. Several clinical terms and controversial disease names have been redefined: eg, the group caused by keratin mutations is referred to by the umbrella term, "keratinopathic ichthyosis"–under which are included epidermolytic ichthyosis, superficial epidermolytic ichthyosis, and ichthyosis Curth-Macklin. "Autosomal recessive congenital ichthyosis" is proposed as an umbrella term for the harlequin ichthyosis, lamellar ichthyosis, and the congenital ichthyosiform erythroderma group. Limitations As more becomes known about these diseases in the future, modifications will be needed. Conclusion We have achieved an international consensus for the classification of inherited ichthyosis that should be useful for all clinicians and can serve as reference point for future research.

https://repository.up.ac.za/bitstream/handle/2263/15323/Oji_Revised(2010).pdf;sequence=1

Revised nomenclature and classification of inherited ichthyoses: Results of the First Ichthyosis Consensus Conference in Sorèze 2009

Objective: To assess the presence of foreign material in the granulomatous cutaneous lesions of patients with systemic sarcoidosis. Design and Setting: Observational study reevaluat- ing histological specimens at a university referral hos- pital. demonstrated in 65 (15.3%) of 425 patients with sys- temic sarcoidosis. In 14 (22%) of the 65 patients, the cu- taneous biopsy specimen showed foreign particles in po- larized light. The skin lesions corresponded to 3 different clinical patterns: an admixture of papules and infiltra- tion of previously undetected minute scars (n=6); scar sarcoidosis (n=4); and subcutaneous nodules (n=4). The lesions were located most frequently in the extremities, involving the knees in 10 patients. Conclusions: The presence of polarizable foreign body material in granulomatous cutaneous lesions is not in- frequent in patients with systemic sarcoidosis. Inocula- tion of foreign matter from a previous inapparent minor trauma may induce granuloma formation in individuals with sarcoidosis. Arch Dermatol. 2001;137:427-430

Foreign Bodies in Granulomatous Cutaneous Lesions of Patients With Systemic Sarcoidosis

The prognostic significance of 20 clinical and histologic parameters was evaluated in a series of 416 laryngeal carcinomas treated between 1969 and 1981 at the Hospital de Santa Cruz y San Pablo, Barcelona, Spain. Clinical follow-up ranged from 2 to 13 years. The variables considered were: age, smoking habits, previous laryngeal disease, duration of clinical symptoms, motility of vocal cords, interval between diagnosis and treatment, postoperative infection, topographic location of the tumor, previous tracheostomy, ulceration, tumor size, resection margins, histologic grading, patterns of growth, types of invasion, mitotic index, depth of invasion, positive lymph nodes, T categories and N categories. Fourteen parameters were found to be significantly related to survival. After a multifactorial study using Cox's life table regression model, 5 of those 14 parameters were found to influence survival independently. The five variables ordered according to prognostic significance were: (1) positive lymph nodes, (2) T categories, (3) histologic grading, (4) ulceration, and (5) location.

Prognostic factors in laryngeal carcinoma: A multifactorial study of 416 cases

Background: Lupus vulgaris (LV) represents the most common form of cutaneous tuberculosis in Europe. However, recent reports from European countries are few and usually limited to isolated cases. Objective: We report the clinical, histopathologic, and bacteriologic data in 10 patients with LV. Methods: The diagnosis of LV was made according to a combination of clinical, histologic, and bacteriologic criteria. In each case a biopsy specimen was obtained for histopathologic study and mycobacteriologic culture. All but one patient received a 9-month course of combined antituberculous therapy. Results: Five patients had evidence of either previous or simultaneous tuberculous foci other than LV. Mycobacterium tuberculosis was isolated from cutaneous lesions in five cases. The histologic study revealed a variable granulomatous reaction, including tuberculoid granulomas, sarcoidlike granulomas, and a "mixed" granulomatous reaction. Conclusion: Although different mechanisms could be implicated in the development of LV, an association with visceral tuberculosis is not infrequent. Combined antituberculous therapy should be the treatment of choice in these patients.

Lupus vulgaris. Clinical, histopathologic, and bacteriologic study of 10 cases.

Background: Diffuse plane xanthoma (DPX) usually has been described in association with a reticuloendothelial disease. However, the incidence of an underlying disease and its relation to clinical and histopathologic features have not been previously described. Objective: We describe the clinicopathologic features of a series of patients with DPX. Methods: Eight patients with DPX were diagnosed and studied between 1987 and 1996. Results: Three of the 8 patients had a reticuloendothelial disease (benign monoclonal gammopathy in 2 and chronic myelomonocytic leukemia in 1). These 3 patients had larger cutaneous lesions and involvement of trunk and extremities. Histologic examination showed only foamy macrophages in 2 of the 8 patients. The remainder exhibited macrophages, foamy histiocytes, lymphocytes, and Touton cells. Conclusion: The incidence of underlying disease associated with DPX seems to be lower than expected. However, patients with DPX should be observed for the potential occurrence of an associated condition. (J Am Acad Dermatol 1998;39:439-42.)

https://www.jaad.org/article/S0190-9622(98)70321-4/pdf

Diffuse plane xanthoma: Clinicopathologic study of 8 cases

Background Cholesterol embolism (CE) is an increasingly common but often underdiagnosed medical problem. The recognition of clinical manifestations of CE is the first step toward a correct diagnosis. Objective Our aim was to characterize the features of CE and the risk factors for fatal outcome. Methods Clinical records of patients with clinical and histopathologic diagnoses of CE seen from January 1993 through March 2003 were reviewed. Results Twenty-six male patients were identified. Mean age was 64 years (range, 48-88 years). All patients had two or more risk factors for atherosclerosis. All but one patient had preexisting symptomatic atherosclerotic disease. At least one precipitating factor was identified in 23 patients (88%). Diagnosis of CE at admission was made in 9 patients only (35%). Cutaneous lesions (88%) and renal failure (73%) were the most common clinical findings. Complications (dialysis, acute pulmonary edema, amputation, or gastrointestinal surgery) were present in 21 patients (81%), and 15 patients died (58%). Previous chronic renal failure was the only variable associated with mortality (relative risk: 4.54, 95% confidence interval 1.26-16.6; P = .02). Limitations The results were obtained from patients admitted to a university hospital. This fact may have selected a higher proportion of severe cases. Conclusions CE was frequently misdiagnosed. Skin lesions were the most common clinical findings and skin biopsy provided histologic confirmation in most of the patients. Chronic renal failure was the only factor related to death.

Abelardo Moreno

Papers

Foreign Bodies in Granulomatous Cutaneous Lesions of Patients With Systemic Sarcoidosis

Prognostic factors in laryngeal carcinoma: A multifactorial study of 416 cases

Lupus vulgaris. Clinical, histopathologic, and bacteriologic study of 10 cases.

Diffuse plane xanthoma: Clinicopathologic study of 8 cases

Cholesterol embolism: still an unrecognized entity with a high mortality rate.