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1.
Chinese Journal of Dermatology ; (12): 480-485, 2022.
Artigo em Chinês | WPRIM | ID: wpr-933586

RESUMO

Objective:To retrospectively analyze the efficacy and safety of dupilumab in the treatment of bullous pemphigoid (BP) .Methods:Clinical data were collected from BP patients who received injections of dupilumab at an initial dose of 600 mg followed by an every-2-week regimen at a dose of 300 mg (the frequency of injections could be increased if necessary) in Department of Dermatology, Peking University First Hospital from October 2020 to October 2021, and their clinical manifestations and changes in laboratory indices were analyzed.Results:A total of 21 BP patients treated with dupilumab were included in this study. Nineteen (90.5%) patients achieved complete or marked disease control after 2-week treatment with dupilumab; 12 patients were followed up for 16 weeks, and all maintained complete disease control at 16 weeks. All patients had a bullous pemphigoid disease area index (BPDAI) score of 122.5 ± 51.1 points at baseline, which decreased to 30.6 ± 27.4 points after 2-week treatment with dupilumab ( t = 8.53, P < 0.001) , and continued to decrease to 12.7 ± 9.1 points after 4-week treatment ( t = 9.73, P < 0.001) . Pruritus was markedly relieved in all the 21 patients within 4-week treatment with dupilumab. Among 10 patients with elevated eosinophil counts at baseline, the eosinophil counts markedly decreased in 9 after treatment. The serum IgE level was elevated in 7 patients at baseline, which markedly decreased in 6 after treatment. Viral conjunctivitis occurred in 1 (4.8%) patient, and no adverse reactions were observed in other patients. Conclusion:Dupilumab is effective in the control of BP and relief of pruritus, with a favorable safety profile.

2.
Chinese Journal of Dermatology ; (12): 676-681, 2022.
Artigo em Chinês | WPRIM | ID: wpr-957722

RESUMO

Objective:To investigate the efficacy and safety of minocycline alone or in combination with low-dose glucocorticoids in the treatment of pemphigus erythematosus and pemphigus herpetiformis, as well as their effect on immune indices.Methods:A retrospective study was conducted, and patients with newly diagnosed pemphigus erythematosus or pemphigus herpetiformis, who received initial treatment with minocycline alone or in combination with low-dose glucocorticoids and were followed up for more than 6 months, were collected from Department of Dermatology, Peking University First Hospital from June 2011 to June 2021. Data on patients′ condition and autoantibody levels were collected at baseline and different follow-up time points, and disease severity, diagnosis, changes in autoantibody levels and their relationships with efficacy were analyzed. The Kaplan-Meier method was used to analyze complete remission rate, and chi-square test to analyze the efficacy among patients with different disease severity or after different treatments.Results:A total of 24 patients of Han nationality were collected and followed up for a median period of 21.8 months, including 15 with pemphigus erythematosus and 9 with pemphigus herpetiformis. The male to female ratio was 1.4∶1, their median age was 68.8 years, and the median duration of disease was 22.1 months. All the 24 patients achieved disease control, and the time to disease control ( M [ Q1, Q3]) was 15.9 (12, 20.1) weeks. Twenty-three (95.8%) patients achieved complete remission, and the time to complete remission was 8.7 (6.4, 10) months. After 1-year treatment, no significant difference in the complete remission rate was observed between patients receiving minocycline monotherapy (11/13) and those receiving combination therapy with low-dose glucocorticoids (9/11, χ2 = 0.16, P = 0.692) . During the follow-up period, 2 patients (8.7%) experienced recurrence in disease control state, 1 of whom achieved complete remission at week 38 after dose adjustment, and the other achieved complete remission after half-a-year treatment with rituximab. There was no significant difference in the efficacy between patients with mild and moderate pemphigus ( χ2 = 0.28, P = 0.599) . Drug-related adverse reactions occurred in 3 cases, including 1 case of tinea corporis on the back and 2 cases of generalized hyperpigmentation of the skin and gingiva. Conclusion:Minocycline alone or in combination with low-dose glucocorticoids was effective for the treatment of mild to moderate pemphigus erythematosus and pemphigus herpetiformis without serious adverse reactions, but long-term efficacy, adverse reactions and patients′ prognosis should be re-evaluated in prospective multi-center studies with a large sample size in the future.

3.
Chinese Journal of Dermatology ; (12): 131-135, 2021.
Artigo em Chinês | WPRIM | ID: wpr-885189

RESUMO

Case 1, a 63-year-old female patient presented with blisters and bullae arising in erythema all over the body with itching for 2 months. Two years ago, the patient underwent peri-intestinal lymph node dissection for the treatment of malignant melanoma of the rectum and anal canal, and received intravenous injection of toripalimab for preventive treatment for 1 year, and generalized skin lesions occurred 2 weeks after drug withdrawal. Direct immunofluorescence testing of erythema on the upper extremities showed that immunoglobulin G (IgG) was deposited along the basement membrane zone; salt-split indirect immunofluorescence testing of a serum sample showed liner deposition of IgG in the epidermis. Enzyme-linked immunosorbent assay (ELISA) revealed that the serum level of anti-BP180 antibodies was over 200 U/ml. The patient was diagnosed with bullous pemphigoid, but it was doubtful whether the disease was caused by PD-1 inhibitor toripalimab or not. Then, the patient received oral minocycline at a dose of 200 mg/d and prednisolone acetate at a dose of 20 mg/d, and topically applied halometasone cream all over the body. After half-a-month treatment, the blisters crusted over and the erythema darkened in color. Case 2, a 36-year-old female patient presented with generalized blisters and itching for more than 3 months. The skin lesions manifested as tense blisters, bullae, bloody bullae and crusts on the edematous erythema. The patient had a 3-year history of vaginal malignant melanoma, which was stage Ⅳ postoperative melanoma with local recurrence and lymph node metastasis nearby the right iliac vessels. After 2-year treatment with intravenous injection of toripalimab, generalized skin lesions occurred all over the body. Direct immunofluorescence testing of the erythema showed weakly positive staining for IgG, and linear deposition of IgG along the basement membrane zone; salt-split indirect immunofluorescence testing of a serum sample showed liner deposition of IgG in the epidermis. ELISA revealed that the serum level of anti-BP180 antibodies was over 200 U/ml. The case 2 was diagnosed with bullous pemphigoid, but it was also doubtful whether the disease was caused by PD-1 inhibitor toripalimab or not. Then, the case 2 was treated with oral doxycycline at a dose of 200 mg/d and oral prednisolone acetate at a dose of 40 mg/d. After 2-week treatment, the blisters completely crusted over and erythema darkened in color.

4.
Chinese Journal of Dermatology ; (12): 279-284, 2020.
Artigo em Chinês | WPRIM | ID: wpr-870273

RESUMO

Objective:To assess the long-term effectiveness and safety of rituximab (RTX) for the treatment of pemphigus, and to evaluate the effect of RTX on immune indices.Methods:A retrospective study was conducted, and patients with pemphigus who received monotherapy or combination therapy with RTX (375 mg/m 2 body surface area, once a week for 4 consecutive weeks) were collected from the Department of Dermatology, Peking University First Hospital from February 2008 to July 2017. Levels of autoantibodies and proportion of B cells in patients were determined at baseline and different follow-up time points, and their changes and relationship with therapeutic effect were analyzed. Time-to-event outcomes (disease control, complete remission and relapse) were estimated using the Kaplan-Meier method. The median ( M) as well as 25th ( P25) and 75th ( P75) percentile values were calculated for repeatedly measured immune indices (autoantibodies and B cells) , and the median level of immune indice-time curve was drawn. Results:A total of 53 patients of Han nationality with pemphigus were included, including 40 with pemphigus vulgaris and 13 with pemphigus foliaceus. The male to female ratio was 0.96∶1, the median age was 37.4 years, and the median duration of disease was 13.4 months at baseline. The median follow-up duration ( P25, P75) was 37.5 (25.0, 54.7) months. Forty-eight (90.6%) patients achieved disease control, and the time to disease control was 1.7 (1.1, 3.2) months. Thirty-eight (71.7%) patients achieved complete remission, and the time to complete remission was 13.1 (9.6, 27.5) months. During the follow up, 12 of the 38 (31.6%) patients who had complete remission experienced recurrence, with the time to recurrence being 12.4 (4.8, 19.8) months. The median immune indice level-time curve showed that anti-Dsg1 and Dsg3 autoantibody levels decreased when skin lesions resolved, but increased when skin lesions relapsed. The most common severe adverse reaction was pulmonary infection, with a mortality rate of 3.8% (2/53) . Conclusions:RTX shows marked long-term effectiveness for the treatment of pemphigus. Pulmonary infection during treatment is worthy of the highest attention. The autoantibody levels can serve as an index for evaluating the effectiveness of RTX in the treatment of pemphigus.

5.
Chinese Journal of Dermatology ; (12): 832-836, 2019.
Artigo em Chinês | WPRIM | ID: wpr-801223

RESUMO

Objective@#To analyze clinicopathological features of parapsoriasis.@*Methods@#Clinical and pathological data were collected from 81 patients with parapsoriasis in Department of Dermatology, Peking University First Hospital between January 2016 and May 2018, and analyzed retrospectively.@*Results@#Among the 81 patients with parapsoriasis, 44 were male and 37 were female, with age ranging from 6 to 77 years. Their disease course ranged from 7 days to 30 years, and the median disease course was 12 months. Moreover, 61 (75.3%) patients were aged less than 40 years, and 20 (24.7%) were aged 41 years and older. Of the 81 patients, 16 (19.8%) were diagnosed with small plaque parapsoriasis, 20 (24.7%) with large plaque parapsoriasis, 37 (45.7%) with pityriasis lichenoides chronica, and 8 (9.9%) with pityriasis lichenoides et varioliformis acuta. Additionally, distribution patterns of lesions included diffuse type in 65 cases (80.2%) , central type in 6 cases (7.4%) , and peripheral type in 10 cases (12.3%) . Histopathological examination of skin lesions revealed liquefaction degeneration of basal cells in 69 cases (85.2%) , migration of lymphocytes into the epidermis in 67 cases (82.7%) , focal parakeratosis in 42 cases (51.9%) , keratinocyte necrosis in 29 cases (35.8%) , extravasation of erythrocytes in 23 cases (28.4%) , epidermal spongiosis in 21 cases (25.9%) , and dermal perivascular focal infiltration in 61 cases (75.3%) .@*Conclusion@#Parapsoriasis has characteristic clinical and pathological manifestations, and a close combination of clinical manifestations with pathological features is necessary for its accurate diagnosis.

6.
Chinese Journal of Pathology ; (12): 100-105, 2015.
Artigo em Chinês | WPRIM | ID: wpr-298143

RESUMO

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunophenotype and gene rearrangement of primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL).</p><p><b>METHODS</b>Seven cases of PCLBCL were enrolled into the study. Clinicopathologic analysis, immunohistochemical staining and gene rearrangement for IgH and Igκ were undertaken in the study.</p><p><b>RESULTS</b>All the seven cases were male, and the median age was 72 years. Patients usually presented with multiple purple tumors, nodules, papules and infiltrative plaques. Two patients had a history of leg injury before onset, and one had mosquito bites. Histologically, the tumor involved the dermis and subcutis with dense and diffuse infiltrative pattern composing of centroblasts and/or immunoblasts. Immunohistochemical staining showed that seven cases (7/7) expressed CD20, six (6/6) expressed bcl-2, four (4/4) expressed MUM-1, four (4/5) expressed CD79a, four (4/5) expressed PAX-5 and four (4/6) expressed bcl-6, respectively. All cases did not express CD3ε, CD45RO, CD10 and CD30. IgH gene rearranged bands were detected in three (3/6) cases and Igκ was detected in one (1/5) case. Six of the seven cases died and the remaining patient, who was 44-year-old, was alive after 22 months of follow-up.</p><p><b>CONCLUSIONS</b>PCLBCL is rare, predominantly affects elderly male patients. PCLBCL has poor prognosis and high mortality, but younger patients seem to have better prognosis. Some cases had a history of trauma or mosquito bites. The relationship between the history and the onset of PCLBCL needs further evaluation.</p>


Assuntos
Idoso , Idoso de 80 Anos ou mais , Animais , Humanos , Masculino , Pessoa de Meia-Idade , Antígenos CD , Culicidae , Rearranjo Gênico , Cadeias Pesadas de Imunoglobulinas , Genética , Cadeias kappa de Imunoglobulina , Genética , Imunofenotipagem , Mordeduras e Picadas de Insetos , Perna (Membro) , Traumatismos da Perna , Linfoma Difuso de Grandes Células B , Genética , Metabolismo , Patologia , Prognóstico , Proteínas Proto-Oncogênicas c-bcl-6 , Metabolismo , Neoplasias Cutâneas , Genética , Patologia
7.
Chinese Journal of Dermatology ; (12): 45-47, 2015.
Artigo em Chinês | WPRIM | ID: wpr-468667

RESUMO

Objective To investigate the effects of pemphigus vulgaris (PV) on pregnancy,childbirth and neonates.Methods Clinical data were collected from 8 pregnant patients with PV who visited the Peking University First Hospital and received follow up.Results Of these patients one developed PV in the third trimester of pregnancy,and the other 7 received treatment for PV and achieved complete subsidence of mucocutaneous lesions before pregnancy.Among the 7 cases,6 were treated with prednisone < 10 mg/d,and 1 was treated with prednisone 22.5 mg/d.Finally,1 patient was lost to follow-up,1 patient underwent artificial abortion on about day 40 of pregnancy with no fluctuation in disease activity.Six patients delivered a normal birth weight baby at term,of whom,1 experienced fluctuation in disease activity in mid-pregnancy,1 suffered from recurrence of PV as a result of drug withdrawal at 2 months after delivery,and the other 4 showed no changes in disease activity.Four out of six neonates were healthy,while two were born with neonatal PV and healed after topical treatment.Conclusions Safe pregnancy and delivery can be achieved in patients with PV whose condition is completely under control with lowdose glucocorticoids after withdrawal of immunosuppressive agents.Although there is a risk of PV in neonates,the prognosis is optimistic.

8.
Chinese Journal of Dermatology ; (12): 788-791, 2013.
Artigo em Chinês | WPRIM | ID: wpr-442118

RESUMO

Objective To analyze the clinical features and treatment of 68 cases of mucous membrane pemphigoid (MMP).Methods Clinical data were collected from 68 patients with MMP at the Department of Dermatology,Peking University First Hospital,between August 1987 and October 2012.Skin manifestations,histopathological and immunological findings were studied,with an emphasis on treatment regimens.Results The two most frequently involved sites were oral (67/68,98.5%) and conjunctival (23/68,33.8%) mucosa in patients with MMP.Immunological examinations included direct immunofluorescence test,indirect immunofluorescence test and enzyme-linked immunosorbent assay,with a positivity rate of 50% (8/16),20.5% (8/39) and 53.7% (22/41) respectively.According to lesion distribution and disease severity,patients were given local therapy (n =5) or low to moderate dose of glucocorticoids (n =55,0.4-0.5 mg/kg or 30 mg per day).The condition was controlled until the dose of glucocorticoids reached 50 mg/d in three patients with cutaneous,oral,ocular,and other mucosal involvement.The time to onset of action of glucocorticoids at the controlling dose was (11.80 ± 5.88) days,and the duration of administration of glucocorticoids at this dose varied from 0.23 to 12 months (average,3.06 ± 2.84 months).Fifteen patients were almost cured,and four patients completely cured.Of the 15 patients almost cured,the time required for a 50% reduction in the dose of glucocorticoids was (13.29 ± 5.76) months,and that required for the control of MMP was (17.33 ± 7.71) months.The dose of glucocorticoids was decreased to 76.5% of the controling dose at 6 months after the control of MMP,and 58.1% of that at 12 months.Oral candidiasis occurred in three patients during the treatment.Conclusions The diagnosis of MMP is mainly based on typical clinical and histopathological findings,and current immunological examinations are insufficiently sensitive.Usually,low to moderate dose of systemic corticosteroids combined with topical therapy can lead to satisfactory treatment outcomes.

9.
Chinese Journal of Dermatology ; (12): 620-623, 2012.
Artigo em Chinês | WPRIM | ID: wpr-424086

RESUMO

Objective To estimate the value of clinical parameters (such as patients' age,longitudinal melanonychia width and location) for the differential diagnosis of malignant and benign longitudinal melanonychia as well as for the evaluation of the necessity for invasive management.Methods A retrospective study was performed on 28 cases of subungual malignant melanoma collected from 2000 to 2010 as well as on 62 cases of benign longitudinal melanonychia from 2005 to 2010.Clinical analysis was carried out to compare the differences in clinical parameters such as.patients' age,longitudinal melanonychia width and lesional location,between the malignant melanoma and benign longitudinal melanonychia cases.Logistic regression analysis and ROC method were used to determine valuable clinical parameters for the differential diagnosis of malignant and benign longitudinal melanonychia.Results Significant differences were observed in the median age at diagnosis (23.0 years vs.52.5 years,Z =5.44,P < 0.01 ),age at onset (21.0 years vs.48.0 years,Z =4.70,P < 0.01 ),and longitudinal melanonychia width (3.0 mm vs.15.0 mm,Z =5.69,P < 0.01 ) between the patients with malignant melanoma and benign longitudinal melanonychia.The involvement of thumb and hallux was observed in 77.8% of the subungual melanoma cases,and 48.3% of the benign cases (x2 =6.57,P < 0.05).ROC method and Logistic regression analysis indicated that the age at onset and diagnosis as well as width of longitudinal melanonychia were of diagnostic value for the differential diagnosis of malignant and benign longitudinal melanonychia.Conclusions Not all longitudinal melanonychia cases need an invasive management at the time of awareness.The age at onset and diagnosis,width of melanonychia and site of the onset appear to be valuable in the differential diagnosis of malignant and benign longitudinal melanonychia,and there is a possibility to guide clinical diagnosis and treatment by establishing a mathematical model with these parameters.

10.
Chinese Journal of Dermatology ; (12): 23-25, 2011.
Artigo em Chinês | WPRIM | ID: wpr-384878

RESUMO

Objective To evaluate the sensitivity and specificity of BP180NC16a-ELISA in the diagnosis of bullous pemphigoid (BP). Methods A multi-center, randomized, double-blind, parallel-controlled study was conducted. Sera were collected from 106 patients with clinically confirmed active BP and 106 control subjects including patients with non-BP bullous diseases, scleroderma, psoriasis or systemic lupus erythematosus,late pregnant women and healthy blood donors. BP180NC16a-ELISA was performed on these sera. The IgG antibody levels measured by ELISA kit were compared with those measured by indirect immunofluorescence (IIF) test. Results Of the 106 BP sera, 81 were positive for BP180NC16a-ELISA with a sensitivity of 76.4%,83 for ⅡF test with a sensitivity of 78.3%. Among the 106 control serum samples, 95 were negative for BP180NC16A-ELISA with a specificity of 89.6%, and 102 for ⅡF test with a specificity of 96.2%. There was no significant difference between the two tests in dignostic sensitivity and specificity for BP (both P > 0.05).Conclusion BP180NC16A-ELISA may serve as an adjuvant tool for the diagnosis of BP.

11.
Chinese Journal of Dermatology ; (12): 452-454, 2010.
Artigo em Chinês | WPRIM | ID: wpr-388629

RESUMO

Objective To investigate the clinical features of skin cancer.Methods Clinical data of skin cancer and precancerous skin lesions confirmed pathologically from 2005 to 2008 in Peking University First Hospital were retrospectively analyzed by using statistical methods.Results A total of 632 cases of skin cancer and precancerous skin lesions were studied.The most common skin cancer was basal cell carcinoma and squamous cell carcinoma (invasive and in situ) which accounted for 29.3%and 24.2%,respectively.The average age at onset was older than 60 years in 55.4%of the patients,between 35 and 59 years in 34.3%,younger than 35 years in 10.3%.The concordance between clinical and pathological diagnosis reached nearly 90.O%for Paget's disease,70.0% for other common skin cancer and precancerous skin lesions.Conclusions Skin cancer and precancerous skin lesions have a predilection for scalp and face.Patients aged from 35 to 59 years account for a significant proportion not only in cutaneous lymphoma but also in melanoma and epithelium-derived nonmelanoma skin cancer.

12.
Chinese Journal of Dermatology ; (12): 777-779, 2010.
Artigo em Chinês | WPRIM | ID: wpr-386130

RESUMO

Objective To evaluate the performance of desmoglein (Dsg)1 enzyme-linked immunosorbent assay (ELISA) in the detection of serum antibodies in patients with pemphigus foliaceus (PF). Methods Sera were obtained from 80 patients with PF and 132 human controls including 33 patients with bullous pemphigoid, 3 patients with linear IgA bullous dermatosis, 2 patients with acquired bullous epidermolysis, 20 patients with systemic lupus erythematosus (SLE), etc, and subjected to a random and blind test by Dsg1 ELISA and indirect immunofluorescence (IIF) on monkey oesophagus. Results The Dsg1 ELISA was positive in 75 (93.8%) patients with PF and 5 (3.8%) human controls (including 1 case of bullous pemphigoid, 1 case of SLE, 1 case of dermatomyositis, 1 case of eczema and 1 normal human control with indeterminate value), and IIF was positive in 71 (88.8%) patients with PF, but in none of the controls. The sensitivity and specificity was 93.8% (95% CI: 0.85 - 0.98) and 96.2% (95% CI: 0.91 - 0.99) respectively for Dsg1 ELISA in the serodiagnosis of PF, 88.8% (95% CI: 0.82 - 0.96) and 100% (95% CI: 0.96 - 1.00) respectively for IIF. There was no statistical difference in the sensitivities (P= 0.289) or specificities (P= 1.000) between the two test methods.Conclusions Dsg1 ELISA is a simple, sensitive and specific serological detection method, and can serve as an adjunct in the diagnosis of PF.

13.
Chinese Journal of Dermatology ; (12)2003.
Artigo em Chinês | WPRIM | ID: wpr-526221

RESUMO

Objective To obtain a better understaning of the clinical features of Castleman tumor associated paraneoplastic pemphigus. Methods The clinical features and therapy of 10 cases of this disease, diagnosed in the Department of Dermatology of Peking University First Hospital were analyzed. Results Castleman tumor was shown to be the most common neoplasm associated with paraneoplastic pemphigus in China. The clinical presentations, histopathologic characteristics, CT scan findings, and immunologic features were all unique. The early diagnosis and removal of the Castleman tumor are crucial for the treatment of this tumor-associated autoimmune disease. Conclusions Because Castleman tumor is directly related to the induction of autoimmunity, early diagnosis and prompt removal of the tumor are essential to the management of this disease.

14.
Chinese Journal of Dermatology ; (12)2003.
Artigo em Chinês | WPRIM | ID: wpr-525817

RESUMO

Objective To identify C0L7Al gene mutations in a family of recessive dystrophic epidermolysis bullosa (RDEB). Methods PCR and direct DNA sequencing were used to determine the mutation sites and types. PCR using allele-specific oligonucleotide primers was performed to further identify the pathogenic cause of this disease. Results The patient examined in this study was a compound heterozygote for a S48P missense mutation in exon 2 and a 3625del 11 PTC mutation in exon 27, which was a novel combination of COL7Al mutations in RDEB. Conclusion The missense mutation and the nonsense mutation in COL7Al gene are underlying causes of the Hallopeau-Siemens variant of RDEB.

15.
Chinese Journal of Dermatology ; (12)1995.
Artigo em Chinês | WPRIM | ID: wpr-673585

RESUMO

Objective To investigate the immunofluoresce nt and immunoblotting features of paraneoplastic pemphigus(PNP).Methods Sera were tested by indirect immunofluorecence(IIF)and immunoblotting(IB)in four patients with PNP.Results Binding of IgGand C3to the intercell ular spaces of epidermis was found in IIF with rat bladder as substrate.The titers decreased after the excision of tumo rs.The autoantibodies also bound to sub-strates of the epithelia of human ski n,rat tongue and esophagus in those p atients.The patients' sera recogni zed 210kD and 190kD antigens from human kera tinocyte extracts with IB analysis.Conclusions IIF with rat bladder as substrate can be used a screening test for PNP.PNP tends to involve epithelia of mucosa.The results of IB test m ay confirm the diagnosis of PNP of the 4p atients.[

16.
Chinese Journal of Dermatology ; (12)1995.
Artigo em Chinês | WPRIM | ID: wpr-521844

RESUMO

Objective To investigate the role of Castleman′s disease in the pathogenesis of paraneoplastic pemphigus (PNP). Methods In six PNP patients associated with Castleman′s disease, routine immunohistochemistry was performed on tumor tissue. Reverse transcription - PCR, DNA sequencing of cloned PCR product and in situ hybridization (ISH) were used to estimate the clonality of the B-cells in the tumors. The expression of the specific tumor B-cell clones was evaluated by Northern blot. Six patients with Castleman′s disease without mucocutaneous lesion and 3 patients with reactive lymphadenopathy were used as the controls. Results Immunohistochemistry showed that CD20-positive B-cells in high density located in lymphoid follicles. The PCR produced one discrete band of about 128 bp in every paraneoplastic pemphigus patients. After sequencing the cloned PCR product, only two kinds of highly homologous sequences were found in all of the PNP patients. The 128 bp sequences were the major clones seen in all patients, and the 122 bp sequences were the relatively minor one seen in 4 patients. Anti-sense RNA probe transcribed from a clone of 128 bp was used in ISH. Signals of ISH located in cytoplasm of the cells in follicles of the tumors. Furthermore, this probe was also used for Northern blot and showed a strong signal in PNP patients. Conclusions Castleman′s disease associated with PNP share a major B-cell clone. The B-cell clone is expressed and maybe produces functional antibody initiating the mucocutaneous immune injury.

17.
Chinese Journal of Dermatology ; (12)1994.
Artigo em Chinês | WPRIM | ID: wpr-522692

RESUMO

Objective To identify the mutation of DKC1 gene and its inheritance in a pedigree with dyskeratosis congenita (DKC). Methods The mutation was detected by polymerase chain reaction(PCR)and DNA sequencing, and restriction endonuclease digestion was performed to confirm the mutation. Results A transition mutation of C to T (1058C-T) in DKC1 gene was found in the proband and his brother. This mutation results in an amino acid change from alanine to valine (A353V) in dyskerin protein. The proband′s mother and sister were carriers of this mutation gene with no phenotype of DKC. Conclusion This pedigree is an X-linked form of DKC with 1058C-T mutation in DKC1 gene.

18.
Chinese Journal of Dermatology ; (12)1994.
Artigo em Chinês | WPRIM | ID: wpr-522017

RESUMO

Objective To identify features of antibodies in the supernatants of cultured Castleman's disease cells.Methods Lymphocytes of Castleman's disease were isolated and cultured.Immunofluorescence and immunoblot assays were performed with IgG extracted from culture supernatants.The immunoglobulin heavy chaingene of cultured tumor B cells was analyzed by RT-PCR,cloning and sequencing.ResultsIg Gextracted from culture supernatant scouldattachtotheepithelialcellsurfacesofmousebladdertissues.Theantibodycouldalsoidentifytwoantigencomponents,210000and190000,ofnormalhumanepidermaltis-sues.ThesequencesimilaritywasfoundinimmunoglobulinheavychaingeneofculturedtumorBcellscom-paredwiththatof6patientswithCastleman'sdiseasepreviouslyreported.Conclusions Castleman's tumor associated with paraneoplastic pemphigus can secret autoantibody with similar features to that found in patients'sera.

19.
Chinese Journal of Dermatology ; (12)1994.
Artigo em Chinês | WPRIM | ID: wpr-520822

RESUMO

0.05).No serious adverse events were reported in these two groups.The incidences of adverse event of mizolastine and loratadine were28.6%and25.5%respectively,there were no statistically significant difference between two groups(? 2 =0.25,P=0.62).Conclusions The efficacy of mizolastine and loratadine is similar in the treatment of CIU,but mizolastine is quicker in action than loratadine.The incidences of adverse events are not different in the two groups.

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