clobetasol and Pemphigoid--Bullous

Bullous pemphigoid (BP) is the most common autoimmune blistering disease. Oral steroids are the standard treatment. We have updated this review, which was first published in 2002, because several new treatments have since been tried.. To assess the effects of treatments for bullous pemphigoid.. We updated searches of the following databases to November 2021: Cochrane Skin Specialised Register, CENTRAL, MEDLINE, and Embase. We searched five trial databases to January 2022, and checked the reference lists of included studies for further references to relevant randomised controlled trials (RCTs).. RCTs of treatments for immunofluorescence-confirmed bullous pemphigoid.. At least two review authors, working independently, evaluated the studies against the review's inclusion criteria and extracted data from included studies. Using GRADE methodology, we assessed the certainty of the evidence for each outcome in each comparison. Our primary outcomes were healing of skin lesions and mortality.. We identified 14 RCTs (1442 participants). The main treatment modalities assessed were oral steroids, topical steroids, and the oral anti-inflammatory antibiotic doxycycline. Most studies reported mortality but adverse events and quality of life were not well reported. We decided to look at the primary outcomes 'disease control' and 'mortality'. Almost all studies investigated different comparisons; two studies were placebo-controlled. The results are therefore based on a single study for each comparison except azathioprine. Most studies involved only small numbers of participants. We assessed the risk of bias for all key outcomes as having 'some concerns' or high risk, due to missing data, inappropriate analysis, or insufficient information. Clobetasol propionate cream versus oral prednisone Compared to oral prednisone, clobetasol propionate cream applied over the whole body probably increases skin healing at day 21 (risk ratio (RR 1.08, 95% confidence interval (CI) 1.03 to 1.13; 1 study, 341 participants; moderate-certainty evidence). Skin healing at 21 days was seen in 99.8% of participants assigned to clobetasol and 92.4% of participants assigned to prednisone. Clobetasol propionate cream applied over the whole body compared to oral prednisone may reduce mortality at one year (RR 0.73, 95% CI 0.53 to 1.01; 1 study, 341 participants; low-certainty evidence). Death occurred in 26.5% (45/170) of participants assigned to clobetasol and 36.3% (62/171) of participants assigned to oral prednisone. This study did not measure quality of life. Clobetasol propionate cream may reduce risk of severe complications by day 21 compared with oral prednisone (RR 0.65, 95% CI 0.50 to 0.86; 1 study, 341 participants; low-certainty evidence). Mild clobetasol propionate cream regimen (10 to 30 g/day) versus standard clobetasol propionate cream regimen (40 g/day) A mild regimen of topical clobetasol propionate applied over the whole body compared to the standard regimen probably does not change skin healing at day 21 (RR 1.00, 95% CI 0.97 to 1.03; 1 study, 312 participants; moderate-certainty evidence). Both groups showed complete healing of lesions at day 21 in 98% participants. A mild regimen of topical clobetasol propionate applied over the whole body compared to the standard regimen may not change mortality at one year (RR 1.00, 95% CI 0.75 to 1.32; 1 study, 312 participants; low-certainty evidence), which occurred in 118/312 (37.9%) participants. This study did not meas. Clobetasol propionate cream applied over the whole body is probably similarly effective as, and may cause less mortality than, oral prednisone for treating bullous pemphigoid. Lower-dose clobetasol propionate cream applied over the whole body is probably similarly effective as standard-dose clobetasol propionate cream and has similar mortality. Doxycycline is less effective but causes less mortality than prednisolone for treating bullous pemphigoid. Other treatments need further investigation.

Topics: Azathioprine; Clobetasol; Dapsone; Doxycycline; Humans; Methylprednisolone; Niacinamide; Pemphigoid, Bullous; Prednisone

Pemphigoid nodularis is a rare form of pemphigoid that joins the clinical picture of prurigo nodularis and the immunological features of bullous pemphigoid, which is therapeutically challenging. Here, we analyze five female patients with a long-lasting course of nodular pemphigoid in terms of clinical and immunological characteristics and therapy. All the patients fulfilled clinical and immunological criteria of nodular pemphigoid. We applied numerous techniques allowing the proper diagnosis: direct and indirect immunofluorescence, salt split skin, ELISA, BIOCHIP, and fluorescence overlay antigen mapping using laser scanning confocal microscopy. Our study showed that 4 of 5 patients fulfilled the clinical and immunological criteria of nodular bullous pemphigoid. Two out of 4 patients presented exclusively nodular lesions; in the other two patients, blisters and erythematous lesions preceded prurigo-like lesions by a few years. The remaining patient had clinical and immunological criteria of nodular mucous membrane pemphigoid, presenting oral erosions, scarring conjunctivitis, and numerous disseminated nodules on the skin. All the patients were treated with multiple medicines; however, it was observed that the use of clobetasol propionate on the entire body plus antidepressants best controlled the disease. Pemphigoid nodularis mainly occurs in elderly women. In cases with coexisting psychological problems, antidepressants should be considered as an important complementary therapy to the basic one with clobetasol propionate.

Topics: Aged; Clobetasol; Female; Follow-Up Studies; Humans; Pemphigoid, Bullous; Prurigo; Skin

Immunotherapy has emerged as a highly effective treatment for numerous cancers. Use of checkpoint inhibitors against various molecules including programmed cell death protein-1 (PD-1), programmed death ligand-1 (PD-L1), and cytotoxic T-lymphocyte-associated protein-4 have become widespread in clinical practice. Compared with conventional chemotherapy, immunotherapy is associated with a unique set of immune reactions known collectively as immune-related adverse events (irAEs). Of known irAEs, cutaneous toxicity is among the most frequently observed in patients treated with immunotherapy. Although often mild, dermatologic toxicity can occasionally be high grade and potentially life-threatening. In this article, we report a case of PD-1 inhibitor-induced bullous pemphigoid-a serious adverse event that has been increasingly observed with use of PD-1/PD-L1 inhibitors. We will also review diagnosis and management of low-grade cutaneous irAEs and bullous disease with checkpoint inhibitors.. PD-1/PD-L1 inhibitor-induced bullous pemphigoid (BP) is a rare but potentially serious dermatologic toxicity associated with checkpoint inhibitorsIn patients with pruritus or rash that is refractory to topical steroids, physicians should have a greater index of suspicion for higher-grade cutaneous immune-related adverse events.There is no standardized treatment algorithm for management of PD-1/PD-L1 inhibitor-induced BP, but patients frequently require topical and systemic steroids.

Topics: Aged; Antineoplastic Agents, Immunological; B7-H1 Antigen; Carcinoma, Non-Small-Cell Lung; Clobetasol; Female; Humans; Immunotherapy; Lung Neoplasms; Nivolumab; Pemphigoid, Bullous; Prednisone; Programmed Cell Death 1 Receptor; Skin

Dermatitis herpetiformis and bullous pemphigoid are bullous autoimmune diseases of the skin microscopically characterized by subepidermal blisters. We present a 77-year-old patient with an 18-month history of disseminated pruritic papular lesions. Direct immunofluorescence microscopy revealed linear deposition of IgG at the basement membrane zone as well as granular deposits of IgA in the papillary dermis. Circulating IgG antibodies against BP180, BP230 and gliadin as well as IgA reactivity against endomysium, tissue transglutaminase, and gliadin were detected compatible with both bullous pemphigoid and dermatitis herpetiformis. Here, we review the English literature on all previously reported patients with co-occurrence of both entities. Interestingly, in previous cases, tissue-bound and serum autoantibodies against the respective target antigens had not yet been completely characterized.

Topics: Aged; Anti-Infective Agents; Anti-Inflammatory Agents; Autoantibodies; Autoantigens; Basement Membrane; Carrier Proteins; Clobetasol; Collagen Type XVII; Cytoskeletal Proteins; Dapsone; Dermatitis Herpetiformis; Dermis; Diet, Gluten-Free; Dystonin; Gliadin; GTP-Binding Proteins; Humans; Immunoglobulin A; Immunoglobulin G; Male; Membrane Glycoproteins; Nerve Tissue Proteins; Non-Fibrillar Collagens; Pemphigoid, Bullous; Protein Glutamine gamma Glutamyltransferase 2; Transglutaminases

Bullous pemphigoid (BP) is the most common auto-immune bullous disorder. Its treatment is difficult due to high age and comorbidities of affected patients.. To assess the effects of treatments for BP.. Randomized therapeutic trials (RCTs) were identified using an automatic search on Pubmed et Embase until March 2009. Large retrospective series with homogeneous therapeutic management were also selected and analyzed.. Forty-four articles were selected and analyzed, which included nine RCTs with a total of 1007 participants (653 patients were included in two trials). Two RCTs comparing different modalities of systemic corticosteroid therapy failed to show differences in measure of disease control. The addition of plasma exchanges (one RCT) or azathioprine (one RCT) allowed to halve the amount of prednisone required for disease control. A further 3-arms RCT compared plasma exchange or azathioprine plus prednisone, but failed to show significant differences for disease control or mortality of BP. One study compared tetracycline plus nicotinamide with prednisolone, no significant difference for disease response was evidenced. A large controlled clinical trial demonstrated that high doses of very potent topical corticosteroids increased initial disease control and 1-year survival of patients with extensive BP, as compared with oral prednisone. Another RCT compared two regimens of potent topical corticosteroids and a non-inferior rate of BP control was obtained with the mild regimen. Finally, a study comparing two immunosuppressant drugs (azathioprine, mycophenolate mofetil) in addition to prednisone failed to show any difference for disease control, recurrence rate or the cumulated doses of prednisone.. Ultrapotent topical corticosteroids (clobetasol propionate; 20 to 40g/day) are effective treatments for BP with fewer systemic side-effects than oral high-dose corticosteroids. Systemic corticosteroids are effective but doses greater than 0.5mg/kg per day are associated with severe side-effects, including decreased survival. The effectiveness of the addition of plasma exchange or immunosuppressants (azathioprine, mycophenolate mofetil) to systemic corticosteroids has not been established. Combination treatment with tetracycline and nicotinamide needs further validation.

Topics: Aged; Azathioprine; Clobetasol; Combined Modality Therapy; Dapsone; Dose-Response Relationship, Drug; Humans; Immunoglobulins, Intravenous; Immunosuppressive Agents; Meta-Analysis as Topic; Multicenter Studies as Topic; Mycophenolic Acid; Niacinamide; Pemphigoid, Bullous; Plasma Exchange; Prednisone; Randomized Controlled Trials as Topic; Recurrence; Retrospective Studies; Tetracycline

Bullous pemphigoid is the most frequent autoimmune bullous skin disease and affects subjects who are about 80 years old. The risk factors are neurological degenerative diseases, poor Karnovski's status and some drugs (aldactone and neuroleptics). Typically, the disease consists of itching eczematous or urticarial sheets, surmounted by blisters. The blisters heal without scars. Mouth and head are rarely involved. The diagnostic is made by histological examination . It shows a subepidermal blister with some degree of dermal infiltrate with lymphocytes and eosinophils. Direct immunofluorescence reveals a linear pattern of IgG deposition along the basal membrane, which signs the diagnosis. Indirect immunofluorescence detecting anti-basal membrane antibodies is of poor diagnostic value. New tests detecting BPAg 2 antibodies by enzyme-linked immunosorbant assay (ELISA) seems to be good markers for disease activity and prognosis. Recommended treatment is topical corticosteroids (clobetasol propionate cream) for several months. It has been showed to be more effective and less dangerous than oral corticotherapy in severe forms of bullous pemphigoid. Corticosteroid sparing agents like methotrexate or mycophenolate mofetil are sometimes used because of cutaneous or systemic side effect of strong and protracted topical corticosteroid therapy. The management of these patients shall be done by specialized and coordinated staff in order to bring the best care.

Topics: Anti-Inflammatory Agents; Autoantigens; Clobetasol; Collagen Type XVII; Enzyme-Linked Immunosorbent Assay; Fluorescent Antibody Technique, Direct; France; Histological Techniques; Humans; Immunoglobulin G; Immunosuppressive Agents; Karnofsky Performance Status; Methotrexate; Mycophenolic Acid; Non-Fibrillar Collagens; Patient Care Team; Pemphigoid, Bullous; Prognosis; Risk Factors

Bullous pemphigoid (BP) is the most common autoimmune blistering disease in the West. Oral steroids are the standard treatment.This is an update of the review published in 2005.. To assess treatments for bullous pemphigoid.. In August 2010 we updated our searches of the Cochrane Skin Group Specialised Register, the Cochrane Central Register of Controlled Trials (Clinical Trials), MEDLINE, EMBASE, and the Ongoing Trials registers.. Randomised controlled trials of treatments for participants with immunofluorescence-confirmed bullous pemphigoid.. At least two authors evaluated the studies for the inclusion criteria, and extracted data independently.. We included 10 randomised controlled trials (with a total of 1049 participants) of moderate to high risk of bias. All studies involved different comparisons, none had a placebo group. In 1 trial plasma exchange plus prednisone gave significantly better disease control at 1 month (0.3 mg/kg: RR 18.78, 95% CI 1.20 to 293.70) than prednisone alone (1.0 mg/kg: RR 1.79, 95% CI 1.11 to 2.90), while another trial showed no difference in disease control at 6 months.No differences in disease control were seen for different doses or formulations of prednisolone (one trial each), for azathioprine plus prednisone compared with prednisone alone (one trial), for prednisolone plus azathioprine compared with prednisolone plus plasma exchange (one trial), for prednisolone plus mycophenolate mofetil or plus azathioprine (one trial), for tetracycline plus nicotinamide compared with prednisolone (one trial). Chinese traditional medicine plus prednisone was not effective in one trial.There were no significant differences in healing in a comparison of a standard regimen of topical steroids (clobetasol) with a milder regimen (RR 1.00, 95% 0.97 to 1.03) in one trial. In another trial, clobetasol showed significantly more disease control than oral prednisolone in people with extensive and moderate disease (RR 1.09, 95% CI 1.02 to 1.17), with significantly reduced mortality and adverse events (RR 1.06, 95% CI 1.00 to 1.12).. Very potent topical steroids are effective and safe treatments for BP, but their use in extensive disease may be limited by side-effects and practical factors. Milder regimens (using lower doses of steroids) are safe and effective in moderate BP. Starting doses of prednisolone greater than 0.75 mg/kg/day do not give additional benefit, lower doses may be adequate to control disease and reduce the incidence and severity of adverse reactions. The effectiveness of adding plasma exchange, azathioprine or mycophenolate mofetil to corticosteroids, and combination treatment with tetracycline and nicotinamide needs further investigation.

Topics: Azathioprine; Clobetasol; Combined Modality Therapy; Drug Therapy, Combination; Drugs, Chinese Herbal; Glucocorticoids; Humans; Immunosuppressive Agents; Niacinamide; Pemphigoid, Bullous; Plasma Exchange; Prednisolone; Prednisone; Randomized Controlled Trials as Topic; Tetracycline

Bullous pemphigoid (BP) is an autoimmune disease with chronic, recurrent bullous eruptions. BP has been reported to be associated with drugs, physical stimuli, malignancies, and immune abnormalities. Its association with renal transplant is rare and only four cases have been reported. We present a case of BP in a 52-year-old man with chronic hepatitis B and C infection who underwent a cadaveric renal transplant 13 years earlier. His graft was still functioning well when BP appeared. The occurrence of BP in our patient might be a result of drugs (furosemide or tacrolimus), viruses, or renal allograft. As the patient was receiving regular T-cell immunosuppressant therapy, his BP lesions were recalcitrant to corticosteroid treatment. We discuss the pathogenesis and treatment of such patients.

Topics: Administration, Cutaneous; Anti-Infective Agents; Clobetasol; Dapsone; Doxycycline; Fatal Outcome; Glomerulonephritis, IGA; Glucocorticoids; Hepatitis B; Hepatitis C; Humans; Kidney Transplantation; Male; Methylprednisolone; Middle Aged; Niacinamide; Pemphigoid, Bullous; Plasmapheresis; Vitamin B Complex

Topics: Adrenal Cortex Hormones; Adult; Age of Onset; Anti-Inflammatory Agents; Clobetasol; Contraindications; Diabetes Complications; Female; Humans; Hypertension; Lichen Planus; Obesity; Ointments; Pemphigoid, Bullous

Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease. Until recently the condition was treated with oral corticosteroids. However, high-dose corticosteroids are poorly tolerated in the elderly and their use has probably contributed to the high mortality rates observed in several cohorts. Accordingly, considerable effort has been directed at identifying corticosteroid-sparing agents, such as immunosuppressant drugs, plasma exchange techniques, intravenous immunoglobulins and tetracycline, that can be used in this clinical setting. Many of these options have appeared to be useful in open series, but they have been found to be ineffective or only marginally effective when tested in randomised, controlled trials. An important breakthrough occurred with the demonstration in a large, randomised trial that a 'super-potent' topical corticosteroid (clobetasol propionate) was not only associated with a significant decrease in severe complications and mortality of BP patients, but was also more effective than oral prednisone. New strategies for BP should include topical clobetasol propionate as first-line therapy in the elderly and consider adjuvant therapy only in the very rare cases that are either resistant or intolerant to this treatment.

Topics: Administration, Topical; Adrenal Cortex Hormones; Aged; Clobetasol; Humans; Pemphigoid, Bullous

Bullous pemphigoid (BP) is the most frequent autoimmune blistering skin disease of the elderly. It is mediated by circulating antibodies directed against two hemidesmosomal proteins of the dermal epidermal junction: BPAG1 and BPAG2. Clinical features consist of pruritus and tense blisters usually surrounded by erythema. Blisters sometimes evolve to erosions, become haemorrhagic or even large erosive areas. Lesions heal without scarring. Lesions are symmetrically located on the thighs, legs, trunck and arms. Mucous membranes are usually uninvolved. Histological examination of a skin biopsy specimen shows a subepidermal blister with eosinophils within the blister and the superficial dermis. Direct immunofluorescence shows linear IgG and/or C3 deposits along the dermal epidermal junction. In France and in Europe, most patients are now treated using topical steroid therapy (clobetasol propionate).

Topics: Aged; Anti-Inflammatory Agents; Autoantigens; Carrier Proteins; Clobetasol; Collagen Type XVII; Cytoskeletal Proteins; Dystonin; Humans; Immunoglobulin G; Nerve Tissue Proteins; Non-Fibrillar Collagens; Pemphigoid, Bullous

Bullous pemphigoid (BP) is the most frequent auto-immune blistering skin disease. Up to recently, it was treated with oral corticosteroids. High dose steroids are poorly tolerated in the elderly and probably contributed to the high mortality rates observed in several cohorts. For years, efforts have been devoted to looking for steroid sparing agents including immunosuppressive drugs, plasma exchanges, intravenous immunoglobulins, tetracycline. Many seemed useful in open series but proved ineffective or marginally effective when tested in randomized controlled trials. An important breakthrough was the demonstration by a large randomized trial that a "super-potent" topical corticosteroid (clobetasol propionate) was not only associated with a significant decrease in severe complications and mortality of BP patients but was also more effective than oral prednisone. New strategies for BP should include topical clobetasol propionate as the first line treatment and consider adjuvant therapy only in the very rare cases that are either resistant to or intolerant of this treatment.

Topics: Administration, Topical; Anti-Inflammatory Agents; Clobetasol; Glucocorticoids; Humans; Immunoglobulins, Intravenous; Immunosuppressive Agents; Pemphigoid, Bullous; Plasma Exchange

Superpotent topical corticosteroids (CS) have been demonstrated to improve bullous pemphigoid (BP) patients' survival. We assessed whether a mild regimen using lower doses of topical CS and a shorter duration could improve the outcome of BP patients even more. Three-hundred and twelve BP patients were included in a multicenter randomized controlled trial and stratified depending on the extent of BP as moderate (n=134) or extensive (n=178). Patients were randomly assigned to the standard regimen (clobetasol propionate cream, 40 g per day initially, with CS tapering over 12 months) or the mild regimen (10-30 g per day), with CS tapering over 4 months. A noninferior rate of BP control was obtained with the mild regimen 156/159 (98%) as compared with the standard regimen 150/150 (100%; P=0.005). Event-free survival, that is, the combined outcome of deaths and life-threatening adverse events did not differ between the two treatment groups (P=0.77). However, upon adjusting through the Cox model for age and Karnofsky score, a strong beneficial effect of the mild regimen was observed in patients with moderate BP, with an almost twofold decrease in the risk of death or life-threatening adverse events relative to the standard regimen (hazard ratio=0.54; 95% confidence interval, 0.30-0.97; P=0.039). This mild regimen allows a 70% reduction of the cumulative doses of CS and improves BP patients' outcome.

Topics: Administration, Topical; Adrenal Glands; Aged; Aged, 80 and over; Clobetasol; Disease-Free Survival; Dose-Response Relationship, Drug; Female; Follow-Up Studies; Glucocorticoids; Humans; Hypothalamo-Hypophyseal System; Male; Pemphigoid, Bullous; Proportional Hazards Models; Recurrence; Treatment Outcome

To identify the prognostic factors of bullous pemphigoid (BP).. Prospective study of patients with BP included in a randomized, controlled trial.. Twenty dermatology departments in France. Patients One hundred seventy patients with BP initially treated with a 40-g/d dosage of clobetasol propionate cream (testing sample) and 171 patients initially treated with oral corticosteroids at a dosage of 0.5 or of 1.0 mg/kg per day, depending on the extent of BP (validation samples).. The end point was overall survival during the first year after BP diagnosis. From the testing sample, associations of clinical and biological variables with overall survival were assessed using univariate and multivariate analyses. Selected predictors were included in a prognostic model. To verify that these predictors were not dependent on the treatment used, the model was then validated independently on the 2 series of BP patients treated with oral corticosteroids.. Median age of the BP patients included in the testing sample was 83 years. The 1-year Kaplan-Meier survival rate was 74%. From univariate analysis, the main deleterious predictors were demographic factors (ie, older age and female sex), associated medical conditions (ie, cardiac insufficiency, history of stroke, and dementia), and low Karnofsky score, which is a measure of the patient's general condition. No factors directly related to BP, in particular extent of cutaneous lesions, were shown to be related to the patients' prognosis. From multivariate analysis, only older age (P = .02) and low Karnofsky score (P<.001) appeared independently predictive of death. From the Cox model including these 2 predictors, the predicted 1-year survival rates were 90% (95% confidence interval [CI], 85%-96%) for patients 83 years or younger with Karnofsky score greater than 40, 79% (95% CI, 69%-90%) for patients older than 83 years with Karnofsky score greater than 40, 65% (95% CI, 50%-86%) for patients 83 years or younger with Karnofsky score of 40 or less, and 38% (95% CI, 26%-57%) for patients older than 83 years with Karnofsky score of 40 or less. Kaplan-Meier survival distributions of patients from the validation samples appeared clearly separated according to these 4 categories and were in close agreement with corresponding predicted 1-year survival rates obtained from the testing sample.. The prognosis of patients with BP is influenced by age and Karnofsky score. These predictors are easy to use and should facilitate the management of BP.

Topics: Administration, Oral; Administration, Topical; Adrenal Cortex Hormones; Analysis of Variance; Cause of Death; Clobetasol; Dose-Response Relationship, Drug; Drug Administration Schedule; Female; Humans; Male; Multivariate Analysis; Pemphigoid, Bullous; Predictive Value of Tests; Probability; Prognosis; Proportional Hazards Models; Prospective Studies; Risk Assessment; Severity of Illness Index; Survival Analysis; Treatment Outcome

Bullous pemphigoid is the most common autoimmune blistering skin disease of the elderly. Because elderly people have low tolerance for standard regimens of oral corticosteroids, we studied whether highly potent topical corticosteroids could decrease mortality while controlling disease.. A total of 341 patients with bullous pemphigoid were enrolled in a randomized, multicenter trial and stratified according to the severity of their disease (moderate or extensive). Patients were randomly assigned to receive either topical clobetasol propionate cream (40 g per day) or oral prednisone (0.5 mg per kilogram of body weight per day for those with moderate disease and 1 mg per kilogram per day for those with extensive disease). The primary end point was overall survival.. Among the 188 patients with extensive bullous pemphigoid, topical corticosteroids were superior to oral prednisone (P=0.02). The one-year survival rate was 76 percent in the topical-corticosteroid group and 58 percent in the oral-prednisone group. Disease was controlled at three weeks in 92 of the 93 patients in the topical-corticosteroid group (99 percent) and 86 of the 95 patients in the oral-prednisone group (91 percent, P=0.02). Severe complications occurred in 27 of the 93 patients in the topical-corticosteroid group (29 percent) and in 51 of the 95 patients in the oral-prednisone group (54 percent, P=0.006). Among the 153 patients with moderate bullous pemphigoid, there were no significant differences between the topical-corticosteroid group and the oral-prednisone group in terms of overall survival, the rate of control at three weeks, or the incidence of severe complications.. Topical corticosteroid therapy is effective for both moderate and severe bullous pemphigoid and is superior to oral corticosteroid therapy for extensive disease.

Topics: Administration, Oral; Administration, Topical; Aged; Aged, 80 and over; Clobetasol; Glucocorticoids; Hospitalization; Humans; Length of Stay; Ointments; Pemphigoid, Bullous; Prednisone; Proportional Hazards Models; Recurrence; Survival Rate

The high mortality at 1 year of patients with bullous pemphigoid is considered to be due mainly to systemic corticosteroids. We report 20 cases of bullous pemphigoid treated solely with class I topical corticosteroid.. Twenty patients with bullous pemphigoid grade 1 and grade 2 were treated with clobetasol propionate 0.05 p. 100 cream at the initial dose of 12 mg/m2/day, progressively tapered over months. Severe forms of bullous pemphigoid covering more than 60 p. 100 of total body area were excluded.. 35 p. 100 of the patients obtained a remission and 35 p. 100 healed (62.5 p. 100 of the mild forms, 16.7 p. 100 of the moderate forms). The average follow up was 11 months after the end of treatment. Side-effects were mild (cutaneous infections, dermal atrophy). Transitory biological anomalies were observed, mainly related to systemic absorption of clobetasol propionate. Some systemic adverse reactions were noted not necessarily attributed to treatment.. Topical steroid treatment was efficient in patients with mild bullous pemphigoid. Our results were comparable to those reported in the literature except for some cutaneous side effects.

Topics: Administration, Topical; Aged; Anti-Inflammatory Agents; Clobetasol; Dose-Response Relationship, Drug; Drug Administration Schedule; Female; Follow-Up Studies; Glucocorticoids; Humans; Male; Ointments; Pemphigoid, Bullous; Prospective Studies; Survival Rate

Super-potent topical corticosteroids (CS) are the mainstay of treatment for bullous pemphigoid. Since super-potent topical CS have systemic effects due to their transcutaneous absorption, we assessed whether super-potent CS were responsible for hydro-saline retention (HSR) in bullous pemphigoid patients.. From 2015 to 2017, patients with newly-diagnosed bullous pemphigoid treated using clobetasol propionate cream at a starting daily dose of 20 to 40 g were subsequently included in a prospective study. HSR was assessed by longitudinally measuring extracellular water (ECW) volume using bioimpedance analysis (BodyStat QuadScan 4000®) from Day 0 to Day 30 after the initiation of topical CS. Other parameters related to HSR such as weight, blood pressure, natriuresis and proteinuria, were also recorded.. Twenty-nine patients (14 men and 15 women) of mean age 81.8 ± 9.3 years were included and analysed. The mean ECW volume decreased from Day 0 to Day 7 (18.1 ± 4.2 vs 16.7 ± 2.7, p = 0.0094) and was maintained from Day 7 to Day 30 (16.8 ± 2.8 vs 17.0 ± 3.4 L; p = 0.8040). Patient weight loss at Day 30 (69.9 ± 13.6 vs 72.5 ± 14.2 kg, p = 0.0085) was closely correlated with the decrease in ECW volume (r = 0.6740, p < 0.0001). No significant changes in natriuresis, 24-hour proteinuria or blood pressure were observed from Day 0 to Day 30.. We found no evidence of HSR in bullous pemphigoid patients treated with super-potent topical CS. Conversely, ECW volume decreased from Day 0 to Day 30, which was correlated with patient weight loss.

Topics: Administration, Topical; Aged; Aged, 80 and over; Clobetasol; Female; Glucocorticoids; Humans; Male; Pemphigoid, Bullous; Prospective Studies

Topics: Clobetasol; Dermatologic Agents; Glucocorticoids; Humans; Pemphigoid, Bullous

Topics: Aged; Autoantibodies; Autoantigens; Autoimmunity; Clobetasol; Cohort Studies; Collagen Type XVII; Cytokines; Dystonin; Enzyme-Linked Immunospot Assay; Glucocorticoids; Humans; Immunoglobulin G; Non-Fibrillar Collagens; Ointments; Pemphigoid, Bullous; Pruritus; T-Lymphocytes, Helper-Inducer; Th17 Cells

Topics: Aged, 80 and over; Cicatrix; Clobetasol; Glucocorticoids; Humans; Leg Injuries; Male; Pemphigoid, Bullous; Recurrence; Time Factors

Dipeptidyl peptidase 4 (DPP-4) inhibitors are increasingly used these days in management of diabetes. There has been reported in a few case reports of increasing association between DPP-4 inhibitor use and bullous pemphigoid (BP). We report a case of association between linagliptin use and BP and subsequent treatment with rituximab.

Topics: Aged; Anti-Bacterial Agents; Clobetasol; Dipeptidyl-Peptidase IV Inhibitors; Drug Eruptions; Humans; Linagliptin; Male; Minocycline; Pemphigoid, Bullous; Prednisone; Rituximab; Treatment Outcome

Bullous pemphigoid (BP) is an autoimmune blistering disease associated with preexisting comorbidities and higher mortality. The interest in using therapy other than oral steroids in BP management results from severe complications and increased risk of death. The efficacy of oral doxycycline or whole-body application of topical clobetasol has been proven in randomized controlled trials. The case series study suggested that combination of tetracycline, nicotinamide, and lesionally administered clobetasol may also be useful.. We conducted a clinical 3-year retrospective study of treatment with tetracycline, nicotinamide, and lesionally administered clobetasol (TNC) in comparison to prednisone (P). Out of 106 patients (mean age 78 ± 9.9 years) with newly diagnosed BP, 59 received tetracycline 1.5 g/daily, nicotinamide 1.2 g/daily, and 0.05% lesionally administered clobetasol cream, and 47 patients - prednisone 0.5 mg/kg daily.. The median time to disease control was achieved after 7 days in both groups. At 4 weeks, 93.2% of patients treated with TNC and 89.1% from P group achieved disease control. The median period between complete remission and relapse was 60 days in the TNC group and 90 days in the P group (P = 0.84). At least one relapse within 1 year was noted in 32.1% of patients from the TNC group and 50% from the P group (P = 0.09). The 1-year survival for the TNC and P groups was 83% and 65.9%, respectively (P = 0.04), and the 3-year survival was 71.2% and 48% (P = 0.019), respectively.. Tetracycline and nicotinamide combined with lesionally administered clobetasol is an alternative, effective treatment with better survival rates compared to prednisone in BP.

Topics: Aged; Aged, 80 and over; Anti-Bacterial Agents; Anti-Inflammatory Agents; Clobetasol; Drug Therapy, Combination; Female; Follow-Up Studies; Humans; Injections, Intralesional; Male; Middle Aged; Niacinamide; Pemphigoid, Bullous; Prednisone; Retrospective Studies; Tetracycline; Time Factors; Vitamin B Complex

Topics: Aged, 80 and over; Autoantibodies; Autoantigens; Clobetasol; Collagen Type XVII; Dermatologic Agents; Drug Therapy, Combination; Dystonin; Humans; Methotrexate; Non-Fibrillar Collagens; Pemphigoid, Bullous; Retrospective Studies; Time Factors

Localized vulvar bullous pemphigoid of childhood is an excessively rare variant of bullous pemphigoid and affects almost exclusively young girls of 7-12 years of age. In contrast to adult-onset bullous pemphigoid, a prompt response to potent topical corticosteroids is observed in the majority of cases, with a favorable prognosis and rare relapses. We report the case of a 7-year-old girl who presented with this condition. Our case reinforces the recognition of this rare subtype of childhood bullous pemphigoid as a distinct entity and enlightens the importance of performing a cutaneous biopsy when clinically indicated.

Topics: Child; Clobetasol; Female; Glucocorticoids; Humans; Pemphigoid, Bullous; Vulvar Diseases

Topics: Administration, Oral; Administration, Topical; Aged, 80 and over; Antibodies, Monoclonal, Humanized; Antineoplastic Agents; Biopsy; Clobetasol; Drug Therapy, Combination; Female; Humans; Leg Dermatoses; Lymphatic Metastasis; Melanoma; Neoplasm Metastasis; Neoplasm Staging; Pemphigoid, Bullous; Prednisone; Skin; Skin Neoplasms

While depletion of circulating autoantibodies using immunoadsorption (IA) is an established therapeutic approach in patients with pemphigus vulgaris, IA has only sporadically been used in other autoimmune bullous disorders. Although bullous pemphigoid (BP) usually responds well to topical and systemic corticosteroids, rapid depletion of serum autoantibodies may be an effective adjuvant treatment option in patients with severe and/or refractory disease.. Case series of 20 patients (13 women, 7 men; mean age 78.6 years; range 56-94 years) with severe or refractory BP. In addition to oral prednisolone (0.25-0.5 mg/kg/day), dapsone (1.0-1.5 mg/kg/day), and clobetasol propionate 0.05 % ointment (lesional application, twice daily), treatment consisted of protein A IA (three sessions on consecutive days). The mean follow-up period was 33.6 months (1-84 months).. The majority of patients showed a rapid and sustained response. One month after treatment, eight patients (42 %; 19 patients were included in the follow-up) were in complete remission; at the last follow-up visit (after 1 to 84 months), that number was 13 (68 %). Not only was there an initial drop in anti-BP180 autoantibodies (by 92 %), the effect also continued after one and three months, with mean autoantibody levels at 26 % and 13 % of baseline, respectively (p < 0.001). Both previously treated and treatment-naive patients showed a significant reduction in anti-BP180NC16A antibody levels throughout the observation period. Adverse events occurred in 13 of the 20 patients (65 %). Three were severe of which two were likely or probably related to IA.. Immunoadsorption is an effective adjuvant treatment option for (the usually elderly) patients with severe and/or refractory BP.

Topics: Aged; Aged, 80 and over; Antimalarials; Autoantibodies; Clobetasol; Dapsone; Female; Glucocorticoids; Humans; Immunosorbent Techniques; Male; Middle Aged; Pemphigoid, Bullous; Prednisolone; Severity of Illness Index; Sorption Detoxification; Staphylococcal Protein A

Topics: Aged; Antibodies, Monoclonal; Antibodies, Monoclonal, Humanized; Clobetasol; Enzyme-Linked Immunosorbent Assay; Female; Glucocorticoids; Humans; Pemphigoid, Bullous; Psoriasis

Iatrogenic Kaposi sarcoma (KS) has previously been reported in patients with bullous pemphigoid (BP), in relation to systemic steroids. To report three cases of previously unreported cutaneous KS during treatment with superpotent topical steroids (STS) and methotrexate (MTX). All patients were elderly men with BP treated with STS for 2 to 32 months (cumulative doses: 2,700-9,150 g) before MTX was introduced (dosage: 10-12.5 mg/week). KS occurred one to nine months after the combined therapy. In one case, KS rapidly resolved after withdrawal of MTX. In two cases, vinblastine and/or radiotherapy were required to achieve regression of KS. Human herpes virus 8 (HHV8) latency-associated nuclear antigen was not expressed in BP lesions biopsied prior to development of KS (n = 3), but HHV8 DNA was detected in BP lesions from the patient with the most aggressive KS. Several predisposing factors were identified, including sex and age, high cumulative doses of STS, combination with MTX, and impaired immune status. In such cases, serum antibodies against HHV8 infection may be investigated in BP patients before introduction of MTX in order to guide clinical monitoring.

Topics: Administration, Cutaneous; Aged; Clobetasol; Drug Therapy, Combination; Humans; Immunosuppressive Agents; Male; Methotrexate; Pemphigoid, Bullous; Sarcoma, Kaposi; Skin Neoplasms

Bullous Pemphigoid (BP) is an inflammatory rare autoimmune bullous dermatosis, which outcome cannot be predicted through clinical investigations. Eosinophils are the main immune infiltrated cells in BP. However, the release of Major Basic Protein (MBP), Eosinophil Derived Neurotoxin (EDN), and Eosinophil Cationic Protein (ECP) upon eosinophil activation has still not been evaluated with respect to BP development. MBP, EDN and ECP were measured by ELISA in serum (n = 61) and blister fluid (n = 20) of patients with BP at baseline, and in serum after 2 months of treatment (n = 41). Eosinophil activation in BP patients was illustrated at baseline by significantly higher MBP, EDN and ECP serum concentrations as compared with control subjects (n = 20), but without distinction according to disease severity or outcome. EDN and ECP values were even higher in the blister fluids (P < 0.01 and P < 0.05, respectively), whereas MBP values were lower (P < 0.001). ECP serum concentration decreased after 60 days of treatment in BP patients with ongoing remission but not in patients who later relapsed (P < 0.05). A reduction of at least 12.8 ng/mL in ECP concentrations provided a positive predictive value for remission of 81%, showing that ECP serum variation could be a useful biomarker stratifying BP patients at risk of relapse.

Topics: Aged; Aged, 80 and over; Anti-Inflammatory Agents; Biomarkers; Case-Control Studies; Cell Movement; Clobetasol; Eosinophil Cationic Protein; Eosinophil Major Basic Protein; Eosinophil-Derived Neurotoxin; Eosinophils; Female; Gene Expression; Humans; Male; Pemphigoid, Bullous; Predictive Value of Tests; Prognosis; Prospective Studies; Recurrence; Remission Induction; Severity of Illness Index; Survival Analysis; Treatment Outcome

Bullous pemfigoid (BP), an autoimmune disorder, can also be induced by some medications. Vildagliptin is a new drug used to treat diabetes mellitus (DM). Recently, a few cases of vildagliptin-induced BP have been described in the literature. We report a patient with BP in which vildagliptin was thought to be as a possible causative agent. The awareness of BP development risk during gliptin therapy can prevent unnecessary usage of systemic drugs with serious side effects.

Topics: Adamantane; Administration, Cutaneous; Clobetasol; Complement C3; Diabetes Mellitus, Type 2; Dipeptidyl-Peptidase IV Inhibitors; Drug Eruptions; Female; Fluorescent Antibody Technique; Gliclazide; Glucocorticoids; Humans; Hypoglycemic Agents; Metformin; Middle Aged; Nitriles; Pemphigoid, Bullous; Pyrrolidines; Skin; Vildagliptin; Withholding Treatment

Topics: Administration, Topical; Betamethasone; Biopsy, Needle; Clobetasol; Disease Progression; Drug Therapy, Combination; Enzyme-Linked Immunosorbent Assay; Epitopes; Fluorescent Antibody Technique, Direct; Follow-Up Studies; Humans; Immunoblotting; Immunohistochemistry; Infant; Japan; Male; Pemphigoid, Bullous; Risk Assessment; Severity of Illness Index; Treatment Outcome

Topics: Administration, Topical; Aged; Clobetasol; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Pemphigoid, Bullous; Tinea; Trichophyton

Current standard of treatment of bullous pemphigoid (BP) is systemic oral corticosteroids (CS). However, significant iatrogenic morbidity and mortality is reported. Studies have shown that topical potent CS is safer than oral prednisolone in BP.. To examine the local and systemic efficacy and adverse effects of whole body clobetasol propionate cream application in patients with mild or severe BP.. Open, clinical records-based retrospective analysis of a series of mild (n = 40) and severe (n = 34) BP patients, treated with ranging doses (20-40 g/day) clobetasol propionate cream. For assessing systemic effects, we observed in selected cases eosinophil count and morning urine cortisol level.. Patients with mild BP achieved in 90.0% disease control and in severe BP in 73.5%. Complete remission was achieved in mild BP in 64.1% (35.9% off therapy and 28.2% on therapy) vs. 41.2% in severe BP (5.9% off therapy and 35.3% on therapy). Local adverse effects were mainly skin atrophy (14.9%) and purpura (5.4%). Systemic adverse effects were rare (n = 3) and consisted of deep vein thrombosis, hypertrichosis and adrenocortical insufficiency. Systemic effect was witnessed by immediate drop of eosinophil count, and decrease in the morning urine cortisol in selected cases.. Topical whole body application of clobetasol propionate cream as monotherapy can be effective and safe in the induction phase of treatment in mild BP and severe BP. When relapse occurs adjuvant systemic medication is mandatory. Potent CS works locally and systemically against BP, at the price of significant local and less significant systemic adverse effects.

Topics: Administration, Topical; Aged; Clobetasol; Female; Glucocorticoids; Humans; Male; Pemphigoid, Bullous; Retrospective Studies; Severity of Illness Index

A 75-year-old Japanese man presented with pruritic blisters and macules on his trunk and extremities. He had been on hemodialysis for 4 years because of chronic renal failure, and in recent months, a polymethylmethacrylate membrane had been used for dialysis. After a change in dialysis membrane to a cellulose triacetate membrane, pruritic tense blisters developed on the extremities in combination with marked blood eosinophilia. Physical examination showed erythematous macules and tense blisters on the trunk and extremities. A biopsy specimen of an erythematous macule showed subepidermal vesicles and eosinophils that attached to the dermal-epidermal junction. Serum level of eosinophilic cationic protein was elevated. From clinical, histological, and immunological findings, a diagnosis of bullous pemphigoid was made. New blisters continued to erupt during the period in which the patient used the cellulose triacetate membrane dialyzer, and even after the use of clobetasol propionate. It resolved only after the patient came back to the use of a synthetic membrane dialyzer. We discontinued the use of clobetasol propionate, and neither bullous eruptions nor blood eosinophilia recurred. These observations suggest that cellulose membrane may be involved in the development of bullous pemphigoid through activation of eosinophils in the blood and the skin lesion, as in the present case.

Topics: Aged; Clobetasol; Humans; Male; Membranes, Artificial; Pemphigoid, Bullous; Polymethyl Methacrylate; Renal Dialysis

Topics: Administration, Topical; Aged, 80 and over; Clobetasol; Genital Diseases, Male; Glucocorticoids; Humans; Male; Pemphigoid, Bullous; Scrotum

Anti-p200 pemphigoid is a recently described autoimmune subepidermal bullous dermatosis characterized by its target antigen and the associated anatomoclinical picture. The treatment is not as yet well defined.. A 73-year-old man consulted for a pruritic bullous eruption with buccal involvement. Direct immunofluorescence revealed linear deposits of IgG and C3 at the dermal-epidermal junction. Elisa screening for circulating anti-BP180 and anti-BP230 antibodies was negative. A diagnosis of bullous pemphigoid was suspected. After an unfavourable clinical outcome under clobetasol and then prednisolone and methotrexate, other immunological tests were performed. Indirect immunofluorescence on NaCl-cleaved skin revealed a deposit of IgG4 antibodies on the dermal side. Immunoblotting showed antibodies directed against a 200-kDa antigen on a dermal extract. A diagnosis of anti-p200 pemphigoid was made. The patient was treated with dapsone combined with prednisolone. Seventy-two hours later, treatment was stopped due to hepatic cytolysis related to immunoallergic hepatitis. Treatment with mycophenolate mofetil was then initiated and resulted in complete remission, which persisted at seven months.. The diagnosis of anti-p200 pemphigoid was made on the basis of a set of clinical and immunological factors. Anti-p200 pemphigoid differs from standard bullous pemphigoid in terms of more frequent cephalic, acral and mucous membrane involvement, as well as a greater degree of miliary scarring. There was no eosinophilia. Elisa screening for anti-BP180 and anti-BP230 antibodies was negative. Immunoblotting showed antibodies directed against a 200kDa protein on dermal extract. The treatment is not well defined, even if dapsone appears to be the most effective therapy. To our knowledge, our patient is the first to be successfully treated with mycophenolate mofetil.. Treatment of anti-p200 pemphigoid is difficult. In our case, treatment by mycophenolate mofetil was effective and could offer an alternative to dapsone.

Topics: Aged; Autoantibodies; Autoantigens; Clobetasol; Dapsone; Drug Resistance; Humans; Immunoglobulin G; Immunosuppressive Agents; Laminin; Male; Methotrexate; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Pemphigoid, Bullous; Prednisolone; Remission Induction; Skin

The value of 230-kDa bullous pemphigoid antibody (BP230) enzyme-linked immunosorbent assay (ELISA) for the diagnosis of bullous pemphigoid (BP) was investigated, but in the immunological follow-up of the disease remains unknown.. Evaluation of BP230 ELISA for diagnosis, follow-up and prediction of relapse in BP.. Monocenter retrospective and prospective study. Patients with typical BP. Detection of autoantibodies by indirect immunofluorescence (IIF), BP180 and BP230 ELISA tests at diagnosis, during the treatment (disease control or failure) and at treatment stop (relapse or not 3 months after).. 74 patients were included. At diagnosis, BP230 ELISA sensitivity was lower than IIF and BP180 ELISA. Combining both ELISA added a weak gain of sensitivity. Both tests paralleled the clinical evolution, especially in case of disease control. At the end of the treatment, BP230 ELISA was not different in patients with or without relapse.. In routine practice, BP230 ELISA does not seem to be a useful additional test in typical BP.

Topics: Aged; Aged, 80 and over; Autoantibodies; Carrier Proteins; Clobetasol; Cytoskeletal Proteins; Dystonin; Enzyme-Linked Immunosorbent Assay; Female; Humans; Male; Membrane Glycoproteins; Methotrexate; Mycophenolic Acid; Nerve Tissue Proteins; Pemphigoid, Bullous; Sensitivity and Specificity; Treatment Outcome

The efficacy of topical steroids in bullous pemphigoid (BP) was assessed by prospective therapeutic trials. Systemic corticosteroids and immunosuppressant agents are indicated in situations of failure or relapses.. To report our experience in the management of BP outside therapeutic trials focusing on the outcome of the patients at 6 and 12 months, compliance and follow-up difficulties.. Monocenter retrospective study with collection of clinical, therapeutic and follow-up data after 6 and 12 months.. Ninety-six patients, mean age 84 years, 65 females, 54% had neurological impairment. The initial dose of clobetasol propionate was 30 g/day, followed by a progressive decrease. In the first 6 months, 14% were lost to follow-up and 17.7% died; 62% were controlled with topical steroids alone, and 25% had adjunctive systemic treatment. Difficulties of compliance were mentioned in 34.4%, without significant difference between controlled and noncontrolled patients. After 12 months, 23% were lost to follow-up and 27.1% died. The mean duration of the treatment was 11.7 ± 8.4 months. After the stop, 18.9% of patients relapsed within 3 months.. We emphasize the frequent recourse to systemic treatments in the first few months, the difficulties of compliance and of follow-up in our day-to-day experience.

Topics: Aged; Aged, 80 and over; Clobetasol; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Lost to Follow-Up; Male; Methotrexate; Middle Aged; Mycophenolic Acid; Patient Compliance; Pemphigoid, Bullous; Recurrence; Retrospective Studies; Treatment Outcome

Topics: Aged; Antibodies, Monoclonal, Murine-Derived; Autoantibodies; Autoimmune Diseases; Clobetasol; Factor VIIa; Factor VIII; Hemophilia A; Hemorrhage; Humans; Immunosuppressive Agents; Male; Methotrexate; Pemphigoid, Bullous; Recombinant Proteins; Rituximab; Treatment Refusal

Types of subepidermal autoimmune bullous dermatosis (AIBD) are classified by anatomoclinical picture and target antigen. A new entity has recently been identified: anti-p200 pemphigoid.. An 82-year-old man consulted for a profuse pruritic bullous eruption refractory to the standard treatments for bullous pemphigoid (BP). Direct immunofluorescence examination of a skin biopsy revealed linear deposits of IgG and of C3 at the dermal-epidermal junction, but Elisa screening for circulating anti-BP180 and anti-BP230 antibodies was negative. Indirect immunofluorescence (IIF) testing of cleaved skin revealed a deposit of IgG4 antibodies on the dermal side. Immunoblotting was negative for a dermal extract but showed an antibody directed against a 200-kD epidermal antigen. A diagnosis of anti-p200 pemphigoid was eventually made and the patient was successfully treated with dapsone.. The diagnosis of anti-p200 pemphigoid was made in this case in spite of discrepancy between the IIF and immunoblotting results, and despite the fact that the target antigen in this disease is considered as being restricted to dermal sites. Anti-p200 pemphigoid usually begins in the second part of life and differs from standard bullous pemphigoid in terms of more frequent mucous membrane and cephalic involvement, as well as a greater degree of miliary scarring. This disease appears more prominent in males and is associated with psoriasis in around one third of cases. Autoantibodies recognize laminin gamma-1, an extra-desmosomal protein that contributes to dermal-epidermal adhesion.. This recently described disease as probably under-diagnosed in France. It should be considered in atypical presentations of bullous disease. Diagnosis is confirmed by immunoblotting detection of autoantibodies directed against a 200-kD antigen normally present in the extract. Dapsone appears to be the most effective treatment.

Topics: Aged, 80 and over; Antibody Specificity; Autoantibodies; Autoantigens; Clobetasol; Complement C3; Dapsone; Epidermis; Fluorescent Antibody Technique, Indirect; Humans; Immunoglobulin G; Immunosuppressive Agents; Laminin; Male; Mycophenolic Acid; Pemphigoid, Bullous; Prednisone

Anti-p200 pemphigoid and bullous pemphigoid (BP) are autoimmune subepidermal blistering diseases characterized by autoantibodies to a 200-kDa dermal antigen (p200) and two hemidesmosomal proteins (BP180 and BP230), respectively. We report a 70-year-old man with haemorrhagic blisters, widespread crusted erosions, and the immunopathological characteristics of anti-p200 pemphigoid. Treatment with doxycycline, topical corticosteroids and immunoadsorption led to rapid clinical remission. However, 19 weeks later, a relapse occurred with generalized itchy urticarial erythema and tense blisters. At this time, both strong dermal and epidermal IgG staining was detected by indirect immunofluorescence microscopy on salt-split skin, and autoantibodies against both p200 and the 16th noncollagenous (NC16A) domain of BP180 were found. Interestingly, the relapse was associated not only with the detection of autoantibodies to a second autoantigen (BP180), but also with an altered clinical phenotype. This case was a unique occasion to directly monitor the emergence of intermolecular epitope spreading during the course of an autoimmune bullous disorder.

Topics: Aged; Anti-Bacterial Agents; Autoantibodies; Autoantigens; Clobetasol; Collagen Type XVII; Doxycycline; Humans; Male; Non-Fibrillar Collagens; Pemphigoid, Bullous; Recurrence; Steroids, Chlorinated; Time Factors; Treatment Outcome

Topics: Aged, 80 and over; Autoantibodies; Autoantigens; Clobetasol; Collagen Type XVII; Colostomy; Complement C3; Eosinophils; Erythema; Female; Humans; Immunoglobulin G; Lymphocytes; Methylprednisolone; Non-Fibrillar Collagens; Pemphigoid, Bullous; Treatment Outcome

Topics: Anti-Inflammatory Agents; Child; Clobetasol; Humans; Lichen Planus; Male; Pemphigoid, Bullous

Pemphigoid vegetans is an exceptionally rare intertriginous variant of bullous pemphigoid characterized by vegetative and purulent lesions present in the groin, axillae, thighs, hands, eyelids and perioral regions. The clinical, histopathological and immunofluorescent profile of a new case of pemphigoid vegetans in a 79-year-old man is reported. Our patient had papillomatous plaques with pustules in the bilateral inguinal folds, which clinically resembled pemphigus vegetans. Also suggesting pemphigus vegetans, an initial skin biopsy showed eosinophilic spongiosis, while a second biopsy showed histological and immunological features diagnostic of pemphigoid. Because only a few cases of pemphigoid vegetans have been reported in the literature, clinical and morphological data are scant. Most reported cases were successfully treated with topical antibiotics or steroids; therefore, appropriate diagnosis of this rare lesion will assist management.

Topics: Aged; Anti-Infective Agents; Anti-Inflammatory Agents; Chlorhexidine; Clobetasol; Fluorescent Antibody Technique; Humans; Immunosuppressive Agents; Male; Pemphigoid, Bullous; Tacrolimus; Trimethoprim, Sulfamethoxazole Drug Combination

Lichen planus pemphigoides (LPP) is a rare, acquired, immunobullous disorder of skin that occasionally involves oral mucous membranes. Clinical, histologic, and immunopathologic findings of the oral manifestations of LPP are described. Clinical features are lichenoid striae, erosions, and ulcerations involving gingiva and buccal mucosae. Histopathologic features are similar to those of ora lichen planus. Direct immunofluorescence demonstrates linear deposits of immunoglobulin G and complement component C3 along the basement membrane with fibrillar deposits of fibrin at the epithelial/lamina propria junction. Fluorescence overlay antigen mapping and laser scanning confocal microscopy of the biopsy specimen exhibits colocalization of in situ antibodies with beta4 integrin, a marker of the keratinocyte basal plasma membrane and upper lamina lucida, consistent with the location of the bullous pemphigoid antigens. This case report describes a case of LPP that presented exclusively as an oral condition. Lichen planus pemphigoides should be considered in the clinical differential diagnosis of vesiculoerosive oral mucosal diseases.

Topics: Anti-Infective Agents; Anti-Inflammatory Agents; Chlorhexidine; Clobetasol; Complement C3; Doxycycline; Female; Fluorescent Antibody Technique; Gingival Diseases; Humans; Immunoglobulin G; Keratinocytes; Lichen Planus, Oral; Microscopy, Confocal; Middle Aged; Mouth Mucosa; Ointments; Oral Ulcer; Pemphigoid, Bullous

Bullous pemphigoid, the most frequent bullous autoimmune dermatosis of the adult, typically presents as disseminated tense blisters on normal or erythematous skin. The diagnosis can be confirmed by direct and indirect immunofluorescence, the detection of circulating autoantibodies against the basement membrane proteins collagen XVII/BP180 and BP230, and histopathology. Autoantibody reactivity against collagen XVII can be measured by ELISA and correlates with disease activity. The ELISA therefore provides a useful tool for monitoring disease activity. Treatment of bullous pemphigoid usually consists of topical and / or systemic steroids in combination with immunosuppressive agents. The intensity of skin involvement and the concurrent diseases and medications of the patient must be considered when selecting a certain treatment. Interdisciplinary cooperation between general practitioners, internists and other specialists facilitates the optimal adaptation of the medication and the early discovery of potential side effects.

Topics: Adrenal Cortex Hormones; Adult; Aged; Anti-Inflammatory Agents; Autoantibodies; Azathioprine; Biopsy; Blotting, Western; Child; Clobetasol; Cyclophosphamide; Dapsone; Dermatologic Agents; Diagnosis, Differential; Drug Therapy, Combination; Enzyme-Linked Immunosorbent Assay; Female; Fluorescent Antibody Technique, Direct; Fluorescent Antibody Technique, Indirect; Humans; Immunoglobulins, Intravenous; Immunosuppressive Agents; Male; Mycophenolic Acid; Niacinamide; Pemphigoid, Bullous; Pregnancy; Skin; Vitamin B Complex

We describe four patients with generalized bullous pemphigoid, who received treatment with oral prednisone and suffered serious corticosteroid-induced iatrogenesis. In all cases, we decided to quickly withdraw the systemic corticosteroid treatment and begin treatment with 0.05 % clobetasol propionate (20 g/day). New outbreaks of pemphigoid or significant side effects were not seen after this change in medication. The topical treatment went on for five to twelve months, and the patients were followed up for extended periods after treatment ended, with no recurrences being observed. Systemic corticosteroid treatment may be replaced by topical treatment without complications, and this may be especially indicated in patients with serious corticosteroid-induced iatrogenesis.

Topics: Administration, Cutaneous; Aged; Aged, 80 and over; Amputation, Surgical; Clobetasol; Comorbidity; Diabetes Mellitus, Type 2; Female; Humans; Hypertension; Iatrogenic Disease; Immunosuppressive Agents; Ischemia; Leg; Pemphigoid, Bullous; Pneumonia; Prednisone; Stroke

Topics: Aged; Anti-Bacterial Agents; Antipruritics; Clobetasol; Female; Humans; Hydroxyzine; Immunosuppressive Agents; Pemphigoid, Bullous; Prednisone; Skin; Tetracycline

Dapsone has been used successfully as adjuvant therapy for bullous pemphigoid (BP). The effectiveness of dapsone for this indication, however, remains controversial. We evaluated the effectiveness and adverse events of dapsone (1.0-1.5 mg/kg per day) in combination with oral methylprednisolone (tapering doses of 0.5 mg/kg per day) and topical clobetasol propionate (initially applied once daily on lesions only) in the treatment of BP. Sixty-two patients treated with this regimen were analyzed retrospectively. Patients were free of new blisters after a mean (+/- SD) of 22 +/- 13 days (median, 20 days). After 3 and 6 months of treatment, methylprednisolone could be reduced to less than 10 mg/d in 71% and 91% of patients, respectively; after 12 months of treatment, 53% of patients were in complete remission without receiving further therapy. Dapsone-related side effects were usually mild except in 3 patients (5%), 2 patients with anemia (hemoglobin level, <7 g/dL) and 1 with agranulocytosis. Our data suggest that dapsone used in combination with systemic and topical corticosteroids may be a relatively safe and effective treatment option for BP.

Topics: Administration, Oral; Administration, Topical; Aged; Anti-Inflammatory Agents; Clobetasol; Dapsone; Drug Therapy, Combination; Female; Follow-Up Studies; Humans; Male; Methylprednisolone; Pemphigoid, Bullous; Recurrence; Remission Induction; Retrospective Studies

Topics: Administration, Topical; Anti-Inflammatory Agents; Clobetasol; Drug Eruptions; Female; Follow-Up Studies; Glucocorticoids; Humans; Pemphigoid, Bullous; Risk Assessment; Treatment Outcome

Topics: Administration, Oral; Administration, Topical; Adsorption; Clobetasol; Glucocorticoids; Humans; Pemphigoid, Bullous; Prednisone

Topics: Administration, Oral; Administration, Topical; Clobetasol; Drug Costs; Glucocorticoids; Humans; Pemphigoid, Bullous; Prednisone

Topics: Administration, Topical; Aged; Anti-Inflammatory Agents; Clobetasol; Cost Control; Diagnosis-Related Groups; Dose-Response Relationship, Drug; Germany; Health Services Accessibility; Humans; Multicenter Studies as Topic; National Health Programs; Pemphigoid, Bullous; Prednisone

Topics: Administration, Oral; Administration, Topical; Autoimmune Diseases; Clobetasol; Glucocorticoids; Humans; Pemphigoid, Bullous; Prednisone; Skin Diseases, Vesiculobullous

Systemic corticosteroid is the main treatment of severe forms of pemphigoid gestationis. We report a case of generalised pemphigoid gestationis successfully treated with very potent topical corticosteroid.. A 37-year-old woman developed during her third pregnancy with a new partner an urticated generalised eruption associated with bullous lesions. The diagnosis of pemphigoid gestationis was confirmed by direct immunofluorescence which detected a linear C3 deposition along the basement membrane zone and the positivity of Herpes Gestationis Factor (10 units). Local treatment with potent corticosteroid (betamethasone dipropionate 0.05 p. 100) failed and the patient was successfully treated by clobetasol propionate 0.05 p. 100 cream. The infant, in good health, was not delivered prematurely.. Severe form of pemphigoid gestationis are currently treated with 0.5 to 1 mg/kg/day of systemic corticosteroids, with maternal and pediatric possible side effects. As in bullous pemphigoid, this observation underlines the efficiency and good tolerance of very potent corticosteroid in severe forms of pemphigoid gestationis.

Topics: Administration, Cutaneous; Adult; Anti-Inflammatory Agents; Betamethasone; Clobetasol; Female; Fluorescent Antibody Technique, Direct; Glucocorticoids; Humans; Pemphigoid, Bullous; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Treatment Outcome

Topics: Administration, Cutaneous; Administration, Oral; Anti-Bacterial Agents; Anti-Inflammatory Agents; Clobetasol; Drug Combinations; Drug Therapy, Combination; Fluorescent Antibody Technique, Direct; Fluorescent Antibody Technique, Indirect; Follow-Up Studies; Glucocorticoids; Humans; Immunoglobulin G; Niacinamide; Oxytetracycline; Pemphigoid, Bullous; Recurrence; Remission Induction; Retreatment

Topics: Administration, Topical; Adult; Aged; Aged, 80 and over; Anti-Inflammatory Agents; Betamethasone Valerate; Clobetasol; Dapsone; Drug Combinations; Drug Tolerance; Female; Follow-Up Studies; Glucocorticoids; Humans; Male; Middle Aged; Pemphigoid, Bullous; Remission Induction; Retrospective Studies; Treatment Outcome

Six patients with pemphigoid and three patients with pemphigus foliaceus were successfully treated with topical corticosteroids. This was especially effective in cases of pretibial localized pemphigoid and in mild cases of bullous pemphigoid and pemphigus foliaceus with negative or low-titer circulating autoantibodies.

Topics: Administration, Topical; Aged; Aged, 80 and over; Anti-Inflammatory Agents; Betamethasone; Betamethasone Valerate; Clobetasol; Dexamethasone; Female; Fluocinonide; Glucocorticoids; Humans; Leg Dermatoses; Male; Middle Aged; Ointments; Pemphigoid, Bullous; Pemphigus; Triamcinolone

Topics: Administration, Cutaneous; Aged; Aged, 80 and over; Anti-Inflammatory Agents; Betamethasone; Clobetasol; Female; Glucocorticoids; Humans; Male; Middle Aged; Pemphigoid, Bullous; Triamcinolone Acetonide

Three patients with bullous pemphigoid have been successfully treated with topical corticosteroids (clobetasol propionate) alone. Another patient required adjuvant systemic corticosteroid therapy. In view of its minimal side effects, we confirm a previous report that topical corticosteroid therapy could represent a valuable alternative in the treatment of limited bullous pemphigoid, although for its extensive forms, such therapy may be incomplete.

Topics: Administration, Cutaneous; Aged; Aged, 80 and over; Clobetasol; Female; Humans; Male; Pemphigoid, Bullous

The potent topical corticosteroid clobetasol propionate was evaluated in an open-label study for its safety and efficacy in the treatment of bullous pemphigoid. Ten patients admitted to the hospital with this diagnosis had clobetasol propionate cream applied twice daily to affected skin until all lesions were healed and for 2 weeks thereafter. Complete epithelialization was achieved in every case within 4 to 17 days of treatment. After discharge, patients received decreasingly less potent corticosteroid creams as maintenance therapy for between 5 weeks and 13 months. As of this report, 7 of the 10 patients remain in remission (range 1 to 10 months). In one patient the disease was exacerbated, and therapy with corticosteroids could not be discontinued. No local or systemic side effects were observed during the study, and plasma cortisol levels were within the normal range when measured during the early tapering-off period while patients were receiving less potent topical corticosteroids. We conclude that clobetasol propionate cream produced rapid healing of bullous lesions without adverse effects and that this treatment followed by maintenance therapy with less potent topical corticosteroids was highly effective in patients with bullous pemphigoid.

Topics: Administration, Cutaneous; Aged; Aged, 80 and over; Betamethasone; Clobetasol; Female; Humans; Male; Middle Aged; Pemphigoid, Bullous; Skin Diseases, Vesiculobullous