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1.

Background:

Pemphigus vulgaris (PV), an autoimmune disorder characterized by blistering skin/mucus membrane lesions, is mediated by desmoglein-3 autoantibodies. We carried out a prospective clinical trial of hematopoietic stem cell transplantation (HSCT) in thymus, bone marrow (BM) and periphery to reconstitute central and peripheral arms of self-tolerance.

Materials and Methods:

Eleven (M:F=5:6) patients with mean age 33.5 years and mean duration of disease 22.8 months, having painful pruritic blisters and ulcers resistant to corticosteroids, were treated with cytokine-stimulated allogeneic HSCT (mean dose: 21.8 × 108 cells/kg BW) from blood group-matched related donors. BM with mean CD34+ count 1.1% was inoculated into thymus, marrow and periphery, followed by two peripheral blood stem cell (PBSC) infusions.

Results:

Recovery began within 24 hours of HSCT and new lesions stopped after 6 months. No graft versus host disease (GvHD)/adverse effect was observed in any patient/donor. Over a mean follow-up of 8.02 years, all patients were well without recurrence/new lesions.

Conclusion:

Drug-resistant PV can be successfully and safely treated by allogeneic HSCT.  相似文献   

2.

Background:

Vitiligo is an acquired depigmenting disorder characterized by the loss of functional melanocytes from the epidermis. Although the etiology of vitiligo is unknown, over the last few years, substantial data from clinical research has greatly supported the ‘Autoimmune theory’ and this is supported by the frequent association of vitiligo with disorders that have an autoimmune origin, including Hashimoto''s thyroiditis, Graves disease, type 1 insulin-dependent diabetes mellitus, and Addison''s disease. As cytokines are important mediators of immunity, there is evidence to suggest that they play a major role in the pathogenesis of autoimmune diseases.

Aim:

Keeping this in view we have assayed sera for cytokine IL-6, IL-2, Tumor necrosis factor (TNF)-α, and IFNγ in 80 cases of vitiligo and compared it with healthy subjects, in order to find out whether they play a role in the pathogenesis of vitiligo or not.

Materials and Methods:

Serum IL-6, IL-2, TNF-α, and IFNγ were done by the indirect enzyme linked immunosorbent assay (ELISA).

Results:

The mean serum IL-6 and IL-2 levels in the patient group were significantly higher when compared with those of the normal controls. The mean serum IFNγ level in patients with vitiligo was significantly lower than that in the control group. There was no significant difference in the serum level of TNF-α between vitiligo and healthy controls.

Conclusion:

An increase in the production of proinflammatory cytokines such as IL-6 and IL-2 in vitiligo patients may play an important role in melanocytic cytotoxicity. Thus, we speculate that the cytokine production of epidermal microenvironment may be involved in vitiligo.  相似文献   

3.
Most varicella-zoster virus (VZV) infections after cord blood transplantation (CBT) present as localized herpes zoster. Here, we report a case of VZV reinfection in an adult patient after CBT that appeared clinically to be varicella. A 50-year-old Japanese man underwent CBT for the management of acute lymphoblastic leukemia. Seventeen months later, he developed a small number of vesicles with umbilicated centers. A skin biopsy showed an intraepidermal blister containing degenerated balloon cells. Subsequently, the skin eruption developed over his entire body. The patient was treated with intravenous acyclovir for 5 days, followed by oral valacyclovir for 9 days. It took more than 3 weeks for most of the skin lesions to scab. Serum levels of anti-VZV IgG on days 3 and 33 after the onset of the skin eruption were negative and 260 mIU/ml, respectively. Serum anti-VZV IgM on days 3 and 33 was not detected. Our patient was diagnosed with VZV reinfection.  相似文献   

4.

Background:

Childhood vitiligo is always a challenge to treat, especially when the disease is progressing rapidly in such a patient. Oral minipulse with betamethasone has been tried in childhood vitiligo and also in some other immune mediated skin disorders with good results.

Aims:

The aim of the present study was to see the overall efficacy of methylprednisolone oral minipulse therapy in combination with topical fluticasone in progressive childhood vitiligo. The combination was tried to achieve a significant amount of repigmentation of vitiligo lesions already present at the initial visit.

Materials and Methods:

Four hundred children with progressive vitiligo were enrolled for this study and were prescribed oral methylprednisolone on two consecutive days every week in a minipulse form for a period of six months. In addition, the patients were instructed to apply fluticasone ointment topically once a day on their vitiligo lesions. The patients were assessed for the remission achieved as well as the extent of repigmentation of their already existent lesions.

Results:

More than 90% of patients went into complete remission after the start of the therapy. Moreover, about 65% (two-thirds) of patients achieved good to excellent repigmentation of lesions at the end of six months of therapy. The therapy was also well tolerated and the side effects seen were almost negligible.

Conclusions:

Oral minipulse treatment with methylprednisolone is an effective treatment option for controlling the disease spread in childhood vitiligo and with the addition of topical fluticasone the extent of repigmentation achieved is also quite significant.  相似文献   

5.
Surgical treatment for vitiligo has been ever evolving. Each surgical modality has its own benefits and limitations. Miniature punch grafting is the most extensively performed surgery, which gives good results in stable vitiligo. Herein we report an unusual type of repigmentation observed after minipunch grafting in a patient of stable vitiligo, which resembled target-like lesions with a “perigraft halo” surrounding individual grafts. Such pigment spread occurred despite the use of 0.5 mm larger graft from the donor site.  相似文献   

6.

Background:

Vitiligo is one of the disorder that has social impact. Both skin and mucous membrane show depigmentation in vitiligo. Depigmentation in oral cavity can be more easily observed and the patient can be given awareness regarding the condition if they are unaware of vitiligo elsewhere in their body and can be guided for treatment.

Aim and objectives:

The aim of this study is to determine the frequency of occurrence of oral mucosal vitiligo in vitiligo patients and to determine the most commonly involved oral mucosal site.

Materials and methods:

The study sample included 100 vitiligo patients. The patients of all age groups and both genders were included. Vitiligo patients associated with systemic conditions such as thyroid disorders, juvenile diabetes mellitus, pernicious anemia, Addison''s disease were excluded in this study.

Results:

Out of 100 vitiligo patients 44 % male and 56% were female. The oral presentation of vitiligo in this study showed depigmentation of buccal mucosa in 5% of patients, labial mucosa in 5% of patients, palate in 8% of patients, gingiva in 2% of patients and alveolar mucosa 1%. Depigmentation of lip was seen in 42% of patients. Lip involvement refers to depigmentation of both the lips or either lip. Also vermilion border involvement was noted in majority of cases. In some cases, the depigmentation of lip extended to the facial skin also.

Conclusion:

In this study 55 patients out of 100 patients showed depigmentation in the oral cavity. Lip involvement was most common in this study showing about 42% of patients. Intraoral mucosal involvement was found in 21% of patients. Among intraoral mucosal site palate was common followed by buccal and labial mucosa, gingiva. Two patients had lip pigmentation as the only manifestation without any depigmentation in the skin.  相似文献   

7.

Background:

Vitiligo is an acquired discoloration of skin and mucous membrane of great cosmetic importance affecting 1-4% of the world''s population. It causes disfiguration in all races, more so in dark-skinned people because of strong contrast. Men, women, and children with vitiligo face severe psychological and social disadvantage.

Aim:

To assess the impact of the disease on the quality of life of patients suffering from vitiligo, also to ascertain any psychological morbidity like depression associated with the disease and to compare the results with that of healthy control group.

Materials and Methods:

Dermatology Life Quality Index (DLQI) and Hamilton Depression Rating Scale (HAMD) are administered to 100 vitiligo patients presenting to the Dermatology OPD and 50 age- and sex-matched healthy controls. Results were analyzed and compared with that of control group. Findings are also correlated in relation to demographic and clinical profile of the disease. Statistical analysis is made to see the significance.

Results:

Vitiligo-affected patients had significantly elevated total DLQI scores (P < 0.001) compared to healthy controls. There is increase in parameters like itch, embarrassment, social and leisure activities in the patient cohort than the control group. Patients of vitiligo are also found to be more depressed (P < 0.001) than the controls.

Conclusion:

Quality of life (QOL) in patients affected with vitiligo declined more severely, and also there is increase in incidence of depression than in the control group. These changes are critical for the psychosocial life of the affected people.  相似文献   

8.
Vitiligo is a common skin disorder, and the pathogenesis is unknown. An increased prevalence of autoimmune thyroid diseases has been described in these patients. The aim of this study is to assess the prevalence of thyroid dysfunction and hypoparathyroidism in patients with vitiligo.

Materials and Methods:

One hundred and nine patients (38 males and 71 females with vitiligo were enrolled. Thyroid physical examination was carried out. Thyroid function tests, thyroid antibodies, calcium and phosphorus were assessed. The collected data were analysed by SPSS version 11.

Results:

Thyromegaly was found in 30.1% of patients. Hypothyroidism was found in 16 (15.7%) out of 109 cases. Two of them had clinical and 14 had subclinical hypothyroidism. One patient had Grave''s disease. Antibody positivity was the most common disorder (anti-TPO and anti-tg were positive in 36.7 and 32.1%, respectively). No patient had hypoparathyroidism.

Conclusion:

According to our study, thyroid dysfunction, particulary hypothyroidism and thyroid antibodies increase in patients with vitiligo. We recommend thyroid antibodies assessment and thyroid function evaluation in these patients.  相似文献   

9.
Adoptive transfer of donor immunity has been demonstrated in animals after bone marrow transplantation (BMT). In humans, several autoimmune diseases have been similarly transferred. Although BMT may, per se, be associated with a modulation of the recipient's immune system, which could trigger or even cause autoimmune diseases, both animal experiments and experience with humans show the likeliness of adoptive transfer of donor immunity to the recipient. We describe a patient with multiple myeloma in whom generalized vitiligo developed within 3 months after allogeneic BMT from his HLA-matched sister with vitiligo. We believe that a form of adoptive transfer of donor immunity to the recipient might play a role in the development of vitiligo. In spite of this, neither de novo development of vitiligo in a genetically predisposed patient nor autoimmune phenomena associated with graft-versus-host disease can be completely excluded as a contributing factor for development of vitiligo in our patient. To our knowledge, this is the first case report of transfer of vitiligo after BMT from a donor with vitiligo.  相似文献   

10.
Stability is taken as the most important parameter before opting for any transplantation technique to treat vitiligo. But, simultaneous donor site repigmentation and depigmentation of grafts at the recipient site has been noted. Similarly donor site depigmentation with complete repigmentation of the recipient area with pigment growing out from each graft has been observed. Successful repigmentation after regrafting in previous punch failure cases has also been reported. Koebner's phenomenon from history (Kp-h) and test grafting were the only available indicators to assess stability. It is quite ironic to note that even after four decades of experience in vitiligo surgery, there seems to be little consensus among workers regarding the optimal required period of stability. Moreover, the exact concept of stability in vitiligo is itself still not transparent and defined beyond doubt Overdependence on KpH or TG may be sometimes misleading in vitiligo. These two reveal the apparent clinical stability only and that may not be the true reflection of stability status of the disease at the molecular level. Antimelanocyte cytotoxic reactivity was observed among CD8+ TCC isolated from perilesional biopsies of patients with vitiligo. An attempt should be made to clearly fathom and define stability, not merely only on clinical ground but along with electron microscopy and histoenzymological analysis of the perilesional and nonlesional skin of vitiligo patients. Probably some growth factors which are responsible for both mitogenic and melanogenic stimulation of melanocytes should also be taken into account. Some serological test(s) could guide us to measure these growth factors.  相似文献   

11.

Background:

Vitiligo is a common, acquired, pigmentary disorder characterized by loss of melanocytes resulting in white spots. This disease carries a lot of social stigma in India.

Objective:

To study the clinico-epidemiological profile of vitiligo patients in Kumaun region of Uttarakhand state in India.

Materials and Methods:

The clinical presentation of vitiligo was examined and analyzed in 762 vitiligo patients attending the Dermatology outdoor of Government Medical College, Haldwani, which is a referral centre for Kumaun region of Uttarakhand state in India.

Results:

Male and female patients were found to be affected almost equally. It was observed that onset of vitiligo was most common in 0-10 years age group, as evidenced by 336 cases out of 762 cases. Acrofacial type of vitiligo (339 cases out of 762) was most commonly observed, followed by vitiligo vulgaris, focal, segmental, mucosal, mixed, and universal vitiligo. The most common site of onset was the lower limbs followed by head and neck, upper limbs, trunk, genitalia, and mucasae. Leucotrichia was observed in 33.5%, Koebner''s phenomenon in 26.3%, and a positive family history in 19% of the vitiligo patients. The other common conditions associated were thyroid disorders (8.9%), diabetes (5.3%), and atopic dermatitis (4.9%).

Conclusion:

The study indicates that acrofacial vitiligo is the most common clinical type observed in Kumaun region of Uttarakhand in India. Onset of vitiligo is most common in first decade of life.  相似文献   

12.

Background:

Suction blister epidermal grafting (SBEG) is one of the most commonly performed types of vitiligo surgery for stable vitiligo. The advantages of SBEG include cost-effectiveness and a relatively easier learning curve for the surgeon.

Aims:

To evaluate the outcome in terms of both recipient and donor site changes, on long-term follow-up of the patients who underwent SBEG in our center.

Methods:

Thirty patients, 21 females and 9 males ages ranging from 9 to 55 years, all having either stable vitiligo not responding to medical line of treatment, were included in the study done which involved a variable follow-up period ranging from 2 to 62 months (mean 23.6, standard deviation 17.79). SBEG was done as day care procedure. The patients were reviewed after 1-week and thereafter followed-up in the subsequent months and years. The results of the procedures were graded as poor (0–24%), fair (24–64%), good (64–94) and excellent (95–100%) depending on the patient satisfaction. Donor site changes were also analyzed.

Results:

The face and lips showed an excellent result and color match and persistent pigment retention. The larger areas, especially the lesions on the limbs showed comparatively less response. Of the total, 6.7% showed poor, 13.3% fair, 30% good and 50% excellent response to treatment. Patient satisfaction wise, 53.3% of the patients were very happy, 26.7% were happy, 10% satisfied and 10% unhappy. Significant positive correlation between patient satisfaction and physician observation was seen (Spearman''s rho 0.866).

Conclusions:

In spite of recent advances in surgical modalities like cellular grafting, SBEG continues to be a good, cost-effective, surgical method of treating vitiligo especially on the face and lip. The donor site also tends to show good healing tendency with minimal scarring and postinflammatory pigmentation.  相似文献   

13.
Ecthyma gangrenosum (EG) is a well-recognized cutaneous infection that most commonly affects immunocompromised patients. It typically occurs on the extremities, or in gluteal and perineal regions. Although Pseudomonas aeruginosa is the most well-known pathogen causing EG, other organisms have been reported to cause EG. Herein we report a rare case of ecthyma gangrenosum presenting as aggressive necrotic skin lesions in perioral and infraorbital areas in a 47-year-old patient with acute myelocytic leukemia after allogeneic bone marrow transplantation. It was caused by Stenotrophomonas maltophilia, which is an aerobic, gram-negative pathogen that has been associated only rarely with cutaneous disease. Blood culture and tissue culture were positive for S. maltophilia. Histological examination revealed numerous tiny bacilli in the dermis and perivascular area. Early recognition of skin lesions caused by S. maltophilia is important to decrease associated mortality in immunosuppressed patients.  相似文献   

14.
银屑病合并急性髓细胞白血病一例的治疗观察   总被引:2,自引:0,他引:2  
目的 报告继发急性髓细胞白血病(M4EO型)的慢性斑块状银屑病1例临床研究及骨髓移植治疗.方法采用临床资料收集,组织病理检查,骨髓和外周血涂片检查,利用流式细胞仪进行细胞免疫分型,用骨髓细胞体外培养做染色体检查及G显带分析.结果患者女,33岁,有寻常性银屑病史20余年,反复出现红斑鳞屑,皮损以斑块为主,有家族史,用多种方法治疗(以中药为主).近来不明原因肌肉酸痛,牙龈出血,发热伴胸骨叩痛.骨髓检查发现异常幼稚单核细胞及早幼粒细胞,并见含有粗大嗜碱颗粒的嗜酸粒细胞,确诊为急性髓细胞白血病M4EO型,经骨髓细胞免疫分型检查符合诊断.骨髓细胞体外培养做染色体检查,+G-显带发现inv(16)的克隆异常和+8克隆异常,染色体核型为46,XX,inv(16)/47,XX,inv(16),+8(2/22).经异基因骨髓移植治疗,银屑病皮损完全消退,急性髓性白血病症状缓解,骨髓异常幼稚细胞减少,病情得到有效控制.结论为国内首例寻常性银屑病(斑块状)继发急性髓细胞白血病M4EO型的临床研究及骨髓移植治疗  相似文献   

15.
Background:Generalized vitiligo is a disease with unpredictable bursts of activity, goal of treatment during the active phase being to stabilize the lesions. This emphasizes the need for a prospective marker for monitoring disease activity to help decide the duration of therapy.Results:The levels of ROS differed significantly between patients and healthy controls, as well as between active versus stable disease groups. In the active disease group, ROS levels were significantly lower in those being treated with systemic steroids than those that were not. ROS levels poorly correlated with disease duration or body surface area involved.Conclusion:A long-term study based on these findings can be conducted to further validate the potential role of ROS in monitoring disease activity vitiligo.  相似文献   

16.

Introduction:

Surgical methods for treatment of vitiligo include punch grafts, blister grafts, follicular grafts and cultured melanocyte grafts. The aim of this study was to determine the efficacy of suction blister grafts for treatment of vitiligo, without the use of phototherapy.

Materials and Methods:

This clinical trial study was conducted on 10 patients with vitiligo that was resistant to usual treatments and with limited involvement in the affected sites. We used cryotherapy and a manual suction device for blistering at the recipient and donor sites, respectively. The blister was separated and fixed with sutures and a dressing to the recipient site. Repigmentation of lesions was evaluated monthly for 6 months after treatment. Repigmentation rates higher than 90%, between 71%–90%, from 51%–70%, and less than 50% were graded as complete, good, moderate, and poor, respectively.

Results:

Ten patients (five females with a mean age of 23.2±3.96 years and five males with a mean age of 30.60±4.15 years) were enrolled in the study. Reponses to treatment after a 6-month follow-up were ‘complete,’ ‘good,’ and ‘moderate’ in 7 (70%), 1 (10%), and 2 (20%) patients, respectively.

Conclusion:

With this technique, patients with restricted sites of involvement, that did not respond to the usual treatments showed very good repigmentation without any additional phototherapy over a 6-month follow-up; moreover, there were no side effects such as scarring.  相似文献   

17.
Background:Vitiligo surgery has come up a long way from punch skin grafts to epidermal cell suspension and latest to the extracted hair follicle outer root sheath cell suspension (EHFORSCS) transplantation. The progressive development from one technique to the other is always on a quest for the best. In the latest development, EHFORSCS, which is an enriched source of follicular inactive melanocyte (melanocyte stem cells), seems to be a good addition to the prevailing cell-based therapies for vitiligo. However, it needs to be explored further in larger, clinical trials.Methodology:A total of 11 patients with sixty stable vitiligo sites attending dermatology outpatient department were included for the open-labeled, prospective, comparative study. The sites were sequentially distributed into two groups of thirty each. Sites of one group were subjected to epidermal melanocyte transfer (EMT) and the others to hair follicular melanocyte transfer (HFMT). Response to treatment was evaluated on the basis of degree of repigmentation; final evaluation of area of involvement was done after completion of 6 months.Results:At the end of 6 months, repigmentation >90% was observed in 83.33% patches of EMT group and 43.33% in HFMT group. Repigmentation >75% was observed in 90% of patches in Group A and 43.34% of patches in Group B, respectively. There was statistically significant difference in the overall pigmentation between these two groups.Conclusion:Both noncultured autologous epidermal cell suspension transfer and noncultured EHFORSCS transfer are safe and effective surgical modalities in the management of stable vitiligo though EMT has shown a better response in the present study. Outer root sheath cell suspension transfer is a novel, minimally invasive technique in its nascent stage in the surgical management of vitiligo which requires further larger clinical trials for evaluation of its efficacy.  相似文献   

18.
Background:Vitiligo is a progressive depigmenting disorder characterized by the loss of functional melanocytes from the epidermis. The etiopathogenesis of vitiligo is still unclear. Heat shock proteins (HSPs) are prime candidates to connect stress to the skin. HSPs were found to be implicated in autoimmune diseases such as rheumatoid arthritis and other skin disorders as psoriasis.Results:Our analysis revealed a significantly higher expression of HSP-70 mRNA in lesional skin biopsies from vitiligo patients compared to nonlesional skin biopsies from vitiligo patients (P < 0.001) and compared to skin biopsies from healthy controls (P < 0.001). The level of HSP-70 was not found to be correlated with age, sex, or disease duration. The expression of HSP-70 was correlated with the disease activity and patients with active vitiligo showed higher mean HSP-70 level compared to those with inactive disease.Conclusions:HSP-70 plays a role in the pathogenesis of vitiligo and may enhance the immune response in active disease.  相似文献   

19.
Childhood vitiligo differs from the adults by showing a higher incidence in females, segmental vitiligo being more common and less frequent association with other systemic autoimmune and endocrine disorders. Childhood vitiligo is often associated with a marked psychosocial and long lasting effect on the self-esteem of the affected children and their parents, hence an adequate treatment is very essential. Treatment of vitiligo is indeed a tough challenge for the dermatologists’ more so in the background of childhood vitiligo. Although multiple therapeutic modalities are available in the therapeutic armamentarium, not all can be used in children. This brief report updates regarding various therapies available in the treatment of childhood vitiligo.  相似文献   

20.

Background:

Mammalian pigmentation results from the synthesis and accumulation of photo protective epidermal melanin. Melanin was formed from the amino acid precursor L-tyrosine within specialized cells, the melanocytes. Oxidative stress has been suggested to be the initial pathogenetic event in melanocyte degeneration with H2O2 accumulation in the epidermis of patients with active disease. Auto immunity has been also suggested as another hypothesis in the pathogenesis of depigmentation disorders. Topical corticosteroids and phototherapy as common treatment modalities have been prescribed in patients with vitiligo. However, they are often not effective and safe (epidermal atrophy). Therefore, research for alternative therapies continues.

Aims:

To evaluate the beneficial effects of a supplementation with antioxidant vitamins (A, C, E) and minerals (zinc, selenium) for vitiligo treatment.

Methods:

Forty experimental autoimmune vitiligo mice C57BL6, aged from 5 to 12 months showing visible signs of induced vitiligo, were sequentially randomized into five parallel groups (8 mice per group). Each group mice was allocated an identical pre coded cage. the first group (SZV) received the ED + 1,4 g zinc (Zn) + 0.04 g selenium (Se) + vitamins (A 118 UI, C 8,5 mg, E 5,4 UI) /kg diet, the second group (PSZV) received the ED + 1,4 g zinc (Zn) + 0.04 g selenium (Se) + vitamins (A 118 UI, C 8.5 mg, E 5,4 UI)/kg diet + Polyphenol orally, the group 3 (PSZ) received the ED + green tea decoction prepared from 100 g/l (polyphenol orally) + 1,4 g Zn + 0.04 g Se, the 4 (P) received the ED + green tea decoction prepared green tea decoction prepared from 100 g/l, the control group 5(C) received the ED + distilled water. Cure was defined as repigmentation of treated sites. Photographic and optical techniques were used both at the baseline and on weekly basis.

Results:

By the end of the study, mices showed visible repigmentation. Using the investigator''s global assessment, therapeutic success in terms of a clear repigmentation documented in 70% of treated mice.

Conclusion:

Our findings suggest that an antioxidant supplementation is significantly beneficial in contributing superior clinical efficacy to cure vitiligo.  相似文献   

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