Evaluation of Clinical Criteria for Diagnosis of Bullous Pemphigoid

Vaillant, L.

doi:10.1001/archderm.134.9.1075

Cited by 171 publications

(143 citation statements)

References 48 publications

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“…bullous pemphigoid (BP) represents the most common cutaneous subepidermal bullous autoimmune disorder. [1][2][3] The disease typically presents in elderly patients with a generalized bullous eruption and, more rarely, mucous membrane involvement. 4,5 Bullous pemphigoid is potentially associated with substantial morbidity.…”

Section: Discussionmentioning

confidence: 99%

A Comparison of Oral Methylprednisolone Plus Azathioprine or Mycophenolate Mofetil for the Treatment of Bullous Pemphigoid

Beissert¹,

Werfel²,

Frieling³

et al. 2007

Arch Dermatol

132

100

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To investigate the safety and efficacy of oral methylprednisolone combined with azathioprine sodium or mycophenolate mofetil for the treatment of bullous pemphigoid.Design: A prospective, multicenter, randomized, nonblinded clinical trial to compare 2 parallel groups of patients with bullous pemphigoid undergoing different treatments.Setting: Thirteen departments of dermatology in Germany.Patients: Patients with bullous pemphigoid (n=73) as evidenced by clinical lesions suggestive of bullous pemphigoid, signs of subepidermal blistering on histologic analysis of skin biopsy specimens, linear deposition of IgG and C3 along the dermoepidermal junction, and deposition of autoantibodies at the blister roof in split-skin analysis.Interventions: Treatment with oral methylprednisolone plus azathioprine (azathioprine group) or oral methylprednisolone plus mycophenolate mofetil (mycophenolate mofetil group). Main Outcome Measures:The cumulative total methylprednisolone doses and rates of remission. Secondary outcome measures were safety profiles and duration of remission.

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Section: Discussionmentioning

confidence: 99%

A Comparison of Oral Methylprednisolone Plus Azathioprine or Mycophenolate Mofetil for the Treatment of Bullous Pemphigoid

Beissert¹,

Werfel²,

Frieling³

et al. 2007

Arch Dermatol

132

100

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“…It mainly occurs in the elderly. Recent European studies reported that a significant proportion of BP patients were in poor general condition and had a poor prognosis [1,2,3,4,5,6]. Neurological disorders such as dementia occurs frequently in the elderly [7].…”

Section: Introductionmentioning

confidence: 99%

Neurological Disorders in Patients with Bullous Pemphigoid

et al. 2007

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Background: Unexpected cases of bullous pemphigoid (BP) have been reported in adult patients with various neurological disorders suggesting a possible relationship between these diseases. Objectives: (1) To determine the prevalence and types of neurological disorders in patients with BP, (2) to assess patients’ functional impairment, and (3) to compare the clinical and biological findings as well as prognosis of BP patients presenting with or without neurological disorders. Methods: BP patients with neurological disorders were selected in a series of 341 consecutive BP patients treated in 20 French Dermatology Departments. Functional impairment was prospectively assessed using the Karnofsky score which is a measure of patients’ general condition. Results: At least one neurological disorder was present in 123 of the 341 BP patients (36%). They primarily consisted of dementia (n = 68; 20%; 95% CI: 16–25%), cerebral stroke (n = 52; 15%; 95% CI: 4–19%), and/or Parkinson’s disease or parkinsonism (n = 32; 9%; 95% CI: 7–13%). BP patients with neurological disease were older than patients without neurological disease (83.8 ± 7.5 years vs. 79.3 ± 10.3 years, p < 10^–4). They also had a lower Karnofsky score (47 ± 19% vs. 74 ± 20%, p < 10^–4). One-year overall survival rates of the two groups were 50.8% (95% CI: 41.8–59.7) and 78.7% (95% CI: 73.0–84.2), respectively (p < 10^–4). In contrast, the number of bullae and main biological features at baseline were not different between the two groups of patients. Conclusion: This study demonstrated a high frequency of neurological disorders, particularly dementia, in BP patients. Most of these patients had a severe functional impairment and a poor prognosis.

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“…We have previously proposed a set of 4 criteria for the diagnosis of BP, including absence of atrophic scars, absence of predominant head and neck involvement, absence of mucosal involvement and age above 70 years [27]. We found that the diagnosis of BP could be made with a sensitivity of 90% and a specificity of 83% if patients had 3 of these 4 criteria [27].…”

Section: Introductionmentioning

confidence: 99%

Clinical Criteria for the Diagnosis of Bullous Pemphigoid: A Reevaluation according to Immunoblot Analysis of Patient Sera

et al. 2004

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Background: We previously proposed a set of 4 clinical criteria for the diagnosis of bullous pemphigoid (BP) that consisted of age greater than 70 years, absence of atrophic scars, absence of mucosal involvement and absence of predominant bullous lesions on the neck and head. These results have been challenged because direct immunoelectron microscopy (IEM), which was used as the standard diagnostic criterion in our initial study, does not identify the different antigens of the basement membrane zone. Objective: To reassess the validity of these clinical criteria for the diagnosis of BP using immunoblot analysis of patient sera as the main diagnostic criterion, in order to precisely identify the antigens recognized by patient sera. Methods: One hundred and eighty-nine sera from patients with various subepidermal autoimmune blistering diseases (AIBDs) were tested by immunoblotting using dermal and epidermal extracts. IEM was used as a complementary diagnostic procedure in a few patients whose serum recognized BPAG2 exclusively or was negative in immunoblotting. Results: 142 patients (75%) had at least 3 of the 4 clinical diagnostic criteria. Sera from patients who lacked the set of BP clinical criteria were more frequently immunoblot negative (34%) than sera from patients who had the criteria (18%; p = 0.025). BPAG1 was more frequently recognized by sera from patients with the set of BP clinical criteria (78%) than by sera from patients without the criteria (45%; p = 5·10^–4). In contrast, BPAG2 was recognized by a great number of sera from patients who lacked the criteria of BP (71%), which was in accordance with the presence of numerous patients with cicatricial pemphigoid in this group. Among patients with various subepidermal AIBDs, the diagnosis of BP could be made with a sensitivity of 86%, a specificity of 90% and an excellent prognostic positive value over 95%, if 3 of these clinical criteria were present. Conclusion: These results confirm the interest of this set of clinical criteria for the rapid diagnosis of BP.

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Evaluation of Clinical Criteria for Diagnosis of Bullous Pemphigoid

Cited by 171 publications

References 48 publications

A Comparison of Oral Methylprednisolone Plus Azathioprine or Mycophenolate Mofetil for the Treatment of Bullous Pemphigoid

A Comparison of Oral Methylprednisolone Plus Azathioprine or Mycophenolate Mofetil for the Treatment of Bullous Pemphigoid

Neurological Disorders in Patients with Bullous Pemphigoid

Clinical Criteria for the Diagnosis of Bullous Pemphigoid: A Reevaluation according to Immunoblot Analysis of Patient Sera

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