Death receptors and apoptosis

Interactions between death receptors from the tumor necrosis factor superfamily and their ligands play a crucial role in the development and the integrity of the epidermis. The major consequence resulting from death receptor targeting is apoptosis. Evidence for dysregulation of death receptor signaling associated with the pathogenesis of selected cutaneous diseases, including toxic epidermal necrolysis, graft versus host disease, and skin cancer, are reviewed herein.     

皮肌炎伴发肿瘤及死亡的临床分析

目的　探讨皮肌炎致死和伴发肿瘤的相关因素。方法　回顾性分析 1990年～ 2 0 0 3年皮肌炎患者的临床资料 ,分组后用 χ2 检验进行统计学分析。结果　年龄、Gottron丘疹、吞咽困难在皮肌炎伴发肿瘤中有显著意义 ;吞咽困难、声音嘶哑、饮水呛咳、肌肉萎缩、肺纤维化、心脏受累在导致皮肌炎 1年内死亡中有显著意义。结论　高龄、Gottron丘疹、吞咽困难预示皮肌炎伴发肿瘤的可能性大 ;吞咽困难、声音嘶哑、饮水呛咳、肌肉萎缩、肺纤维化、心脏受累提示皮肌炎预后不佳     

Death receptors in cutaneous biology and disease

Death receptors are a growing family of transmembrane proteins that can detect the presence of specific extracellular death signals and rapidly trigger cellular destruction by apoptosis. Expression and signaling by death receptors and their respective ligands is a tightly regulated process essential for key physiologic functions in a variety of organs, including the skin. Several death receptors and ligands, Fas and Fas ligand being the most important to date, are expressed in the skin and have proven to be essential in contributing to its functional integrity. Recent evidence has shown that Fas-induced keratinocyte apoptosis in response to ultraviolet light, prevents the accumulation of pro-carcinogenic p53 mutations by deleting ultraviolet-mutated keratinocytes. Further- more, there is strong evidence that dysregulation of Fas expression and/or signaling contributes to the pathogenesis of toxic epidermal necrolysis, acute cutaneous graft versus host disease, contact hypersensitivity and melanoma metastasis. With these new developments, strategies for modulating the function of death receptor signaling pathways have emerged and provided novel therapeutic possibilities. Specific blockade of Fas, for example with intravenous immunoglobulin preparations that contain specific anti-Fas antibodies, has shown great promise in the treatment of toxic epidermal necrolysis and may also be useful in the treatment acute graft versus host disease. Likewise, induction of death signaling by ultraviolet light can lead to hapten-specific tolerance, and gene transfer of Fas ligand to dendritic cells can be used to induce antigen specific tolerance by deleting antigen-specific T cells. Further developments in this field may have important clinical implications in cutaneous disease.     

Factors Responsible for Death in Patients with Pemphigus

The records of 10 patients who died with pemphigus have been examined for factors affecting and contributing to death. Early initiation of therapy, the age at onset of the disease, and the mode of administration of corticosteroids, conventional or in pulse form, did not affect the survival. The cutaneous involvement was extensive in all 10 patients; it ranged between 30-80%. Septicemia was the commonest event preceeding death; in 4 cases, it was due to Staphylococcus aureus.     

Death from colonic disease in epidermolysis bullosa dystrophica

Background

Squamous cell carcinomas and renal failure were reported the causes of death in patients with recessive dystrophic epidermolysis bullosa (RDEB). Death from colonic disease in epidermolysis bullosa (EB) is never reported.

Case presentation

We demonstrate a male patient with RDEB. He suffered megacolon due to fecal impaction and died from sigmoid colon perforation with peritonitis at age 35 years.

Conclusion

Constipation is a common clinical feature of RDEB, but fetal complications of chronic constipation are rarely reported. To the author's best knowledge, it has not been reported or recognized in the English literature previously. The aggressive assessment of constipation with fecal impaction is recommended in patients with RDEB.     

皮肤病住院患者死亡原因分析

目的:探讨皮肤病住院患者死亡病例的死亡原因。方法:收集 1985年－2000年我院皮肤病患者死亡病例23例,对其致死原因和相关因素进行分析。结果:致死原因依次为感染,呼吸衰竭,合并肿瘤以及心脏病变等。结论:皮肤病住院患者死亡病例中感染是导致死亡的首要原因。     

天疱疮160例临床分析以及死亡病例原因分析

目的总结天疱疮的临床特点、治疗方法及死亡原因。方法对161例天疱疮临床资料进行回顾性分析。结果常见诱因和加重因素为不规则使用激素和精神因素,抗Dsg1/3的阳性率为89%,轻、中、重型的激素使用量和住院天数差异有统计学意义,死亡常见于寻常型和重型天疱疮,肺炎为死亡常见的原因。结论规则使用激素和加强患者心理教育可减少疾病复发,抗Dsg1/3可作为天疱疮常规检查,病情分型影响激素的用量,感染是死亡的常见原因。     

系统性红斑狼疮死亡相关因素的分析

目的：探讨系统性红斑狼疮(SLE)患者死亡的相关因素。方法：收集1998年1月至2004年5月在我院住院的SLE患者206例，其中存活者168人，死亡者38人，利用χ^2检验，对死亡相关因素进行对比分析。结果：SLE患者死亡的高危因素是合并感染、肾损害、心血管损害、神经精神症状；补体C3水平下降也与SLE患者死亡存在相关关系。结论：合并感染是导致SLE患者死亡的首要因素。