类脂质渐进性坏死的研究进展

类脂质渐进性坏死是发生在真皮结缔组织的疾病,以小腿胫前的硬皮病样斑块为临床特征.其确切的发病机制不清楚,可能与糖尿病、免疫机制、胶原变性、血小板异常及创伤等多种因素有关.类脂质渐进性坏死同前尚无有效的治疗方法,糖皮质激素、免疫治疗、抗肿瘤坏死因子药物、光疗、延胡索酸酯及外科手术等有一定的疗效.     

类脂质渐进性坏死研究进展

类脂质渐进性坏死是一种少见的慢性肉芽肿性疾病,本文综述了其流行病学,发病机制,包括血管、胶原蛋白、免疫球蛋白、中性粒细胞和遗传学,并总结了类脂质渐进性坏死的治疗方法。     

Cutaneous anaesthesia in necrobiosis lipoidica

Cutaneous sensation in plaques of necrobiosis lipoidica was assessed in five non-diabetic and seven diabetic patients. Eleven of the twelve showed partial or complete anaesthesia of the affected skin. These findings are important in the differential diagnosis of tuberculoid leprosy. Further studies of nerve function in cutaneous granulomas need to be carried out.     

Transepithelial elimination in necrobiosis lipoidica

Elimination of necrotic material through the hair follicle as a means of excretion has been observed in three cases of necrobiosis lipoidica. Clinically this process presents as comedo-like plugs localized mainly in the periphery of the lesions.     

类脂质渐进性坏死一例报告

类脂质渐进性坏死较为少见,现将笔者所见一例报告如下.     

类脂质渐进性坏死1例

报告类脂质渐进性坏死1例。患者女,68岁,因右下肢胫前斑块2年就诊。皮肤专科检查:右下肢胫前可见一大小约10 cm×3 cm黄棕色斑块,边界清楚,表面光滑有光泽,可见毛细血管扩张和褐色斑,上覆鳞屑,上下端皮损中央萎缩凹陷,上见痂,质硬,无压痛。皮损组织病理检查:表皮轻度萎缩,真皮见胶原变性灶,周围组织细胞呈栅栏状排列,真皮浅深层毛细血管增生,管壁增厚,血管周围淋巴细胞、浆细胞、组织细胞及多核巨细胞浸润。实验室检查:血、尿、大便常规、肝肾功能正常,口服葡萄糖耐量试验提示糖耐量异常,糖化血红蛋白正常。诊断:类脂质渐进性坏死。予铒点阵激光(2 940 nm)联合卤米松/三氯生乳膏外用,皮损较前消退,目前仍在随访中。     

Transcutaneous oxygen partial pressure measurement in follow-up of patients with erysipelas

In 24 patients with erysipelas, skin oxygen tension (tcPO2) was measured in the centre, at the border and outside the area of skin inflammation before, during and after antibiotic treatment. At the same time ESR, leucocytes and body temperature were determined. Skin erythema persisted over a period of about 7 days. Leucocytes and body temperature showed normal values after 3 days, while ESR was raised continuously in the first 3 weeks. Skin oxygen tension showed a good correlation with the clinical picture, but was still reduced at the end of therapy. About 2 weeks after disappearance of the clinical signs of inflammation, tcPO2 values were back to normal. The measurement of transcutaneous oxygen pressure can supplement the classic parameters of inflammation as a valuable tool for follow-up examinations in patients with erysipelas.     

Clofazimine--therapeutic alternative in necrobiosis lipoidica and granuloma anulare

Twenty patients, ten suffering from disseminated granuloma anulare and ten from necrobiosis lipoidica, were treated with clofazimine 200 mg p.o. daily. Six patients in each group (60%) responded to this regimen, and three of the responders in each group achieved complete remission of the dermatosis. In eight patients (40%) no improvement at all was observed. All the patients treated had reddening of the skin, but this was reversible after the end of therapy, as were the other side-effects, i.e. diarrhoea and dryness of the skin, which were not experienced by all patients.     

穿通型类脂质渐进性坏死一例

患者男,24岁。因四肢及腹部起红色斑块,周边隆起3年就诊。3年前无明显诱因右下肢胫前出现1元硬币大小红色斑块,微痒,未曾治疗,皮损逐渐增大、增多,渐发展至腹部,偶有痛痒感。既往有1型糖尿病病史10年,口服降糖药及注射胰岛素治疗（具体不详）,血糖控制不稳。否认高血压、心脏病等内科疾病,否认肝炎、结核等传染性疾病,否认药物过敏史……     

Update: Treatment of necrobiosis lipoidica

Necrobiosis lipoidica (NL) is a rare granulomatous disease of hitherto unclear etiology frequently seen in patients with diabetes. Characterized by its potential for ulcerations, it often presents a serious burden for those affected. There are currently neither German nor European guidelines for the treatment of NL. At the same time, standard treatment with topical or intralesional corticosteroids does not always show satisfactory results. We therefore set out to evaluate whether the various treatment regimens published since 2000 have actually expanded the therapeutic armamentarium in a relevant manner. Included were all publications that described more than one patient being treated with any given therapeutic modality. Overall, we analyzed data for 16 different treatment regimens reported in 49 publications. The largest amount of data exists for topical PUVA therapy, photodynamic therapy (PDT), and systemic treatment with fumaric acid esters. Remarkably, our analysis showed that with an increase in the number of documented patients treated with a given therapeutic modality, the proportion of those achieving a complete or partial response actually decreased. This was interpreted as publication bias. Thus, no clear recommendation can be given for second‐line therapy in case topical or intralesional corticosteroids fail.     

Venous insufficiency in patients with necrobiosis lipoidica

The pretibial area is the most frequently affected site in necrobiosis lipoidica (NL), but proposed mechanisms of NL cannot fully explain this high frequency. Although a few case reports indicate NL patients are complicated with venous insufficiency, no accurate assessment of the relationship between these two conditions has been performed. By using color Doppler ultrasonographic screening of four NL patients for venous insufficiency, we detected venous insufficiency in at least one leg of each patient. NL lesions were observed on all legs with venous insufficiency, and laboratory examination findings revealed that all the patients had hypercholesterolemia. The skin lesions did not respond satisfactorily to 6-month use of anticholesterolemic medication and elastic stockings. However, these results indicate that both hyperlipidemia and venous reflux, in addition to other pathogenic factors, can trigger tissue damage in the lower legs and lead to the onset of NL.     

Long-term results of topical PUVA in necrobiosis lipoidica

The aim of our study was to evaluate the long-term results of topical psoralen ultraviolet A (PUVA) in patients with necrobiosis lipoidica (NL), in whom conventional methods (pentoxifylline, vitamin E, tretinoin, and topical or intralesional corticosteroids) had failed. The study comprised 10 women (age range 17-44 years), six of whom were insulin-dependent diabetics and four were diabetes-free. Duration of NL ranged from 3 to 10 years. The patients were treated with a 0.005% aqueous solution of 8-methoxypsoralen, applied topically for 30 min, and subsequently irradiated with UVA three times weekly. All the patients experienced almost complete remission (softening of skin lesions, no hyperpigmentation, lack of lesion progression) after a mean of 47 sessions (mean UVA cumulative dose 69.5 J/cm2). They were followed up for 12-24 months, during which time two recurrences, both in diabetic patients, were observed after 8 and 12 months of treatment cessation, which further resolved after another course of topical PUVA. We conclude that topical PUVA is well tolerated by NL patients and may serve as an alternative therapeutic regimen.