National audit on the management of bullous pemphigoid

Abstract

Summary Background Bullous pemphigoid (BP) is an autoimmune, subepidermal, blistering condition that typically affects elderly people. Aim To undertake a national clinical audit based on standards derived from the British Association of Dermatologists (BAD) clinical guidelines on the management of BP. Methods In 2018, BAD members were invited to submit data for five consecutive adults with BP per centre, who had been under hospital supervision for at least 12 months, in a national audit over an 11‐week period. Results In total, 123 responders from 120 hospitals provided data for 524 cases. Diagnosis was made either clinically (10.7%; 56 of 524) or through histology with direct immunofluorescence (41.6%; 218 of 524), indirect immunofluorescence (10.3%; 54 of 524) or both (37.4%; 196 of 524). Most patients had very mild baseline disease (63.9%; 225 of 352) with 21.9% (77 of 352) considered mild, 9.8% (31 of 352) moderate and 5.4% (19 of 352) severe. Documentation of diabetes, glycated haemoglobin (HbA1c), blood pressure and hypertension was available for 54.1% (283 of 523), 51% (267 of 524), 44.2% (231 of 522) and 61.5% (321 of 522) of cases, respectively. Oral corticosteroids were commenced in 85.5% (448 of 524) of patients, with 38.4% (172 of 448) of these having documented risk of osteoporosis; data regarding prescription of bone‐protection therapies were available for 99.7% (447 of 448) of cases, with 75.6% (338 of 447) of these having a bone‐protection prescription. Patient satisfaction was documented in 59.3% (310 of 523) of cases. Systemic treatment was commenced in 95.9% (502 of 524) of cases during the 12‐month assessment period, with baseline blood test and follow‐up data available for 96.6% (485 of 502) and 95.6% (480 of 502), respectively. Documentation of baseline blood tests was available for 87.4% (424 of 485) of cases, with follow‐up tests recorded in 69.8% (335 of 480). Conclusion Overall, compliance with elements of documentation was moderate or low, whereas standards pertaining to direct care were high.

Osteoporosis in Skin Diseases

“…In line with this, sustained or enhanced serum levels of IL-17 were associated with relapses in BP [93]. Therefore, the marked association between OP and pemphigus can be explained at the molecular level by the immunopathogenic process and by the pro-inflammatory cells and cytokines shared in both disorders that lead to a net increase in bone resorption [92]. The guidelines for the treatment of BP of the British Association of Dermatologists recommend topical steroids as the first line of treatment if the pathology is limited; in the case of generalized pathology, the first line to be considered are topical steroids or high doses of systemic cortisone, while immunosuppressants (methotrexate, mycophenolate mofetil, azathioprine and dapsone) are to be considered the second line of treatment in extensive pathology, in patients who do not respond to oral corticosteroids or who have contraindications to it [92].…”

“…These considerations led the authors to argue that these dermatological patients may benefit from increased screening for OP and interventions to prevent fractures [91]. Pemphigus is an autoimmune disease dependent on T cells principally of self-reactive Th2 cells, which also involves pro-inflammatory cytokines of the innate immune system, such as TNF, IL-1, and IL-6 [92]. Moreover, Le Jan et al [93] identified IL-17 and related cytokines such as IL-22 and IL-23 in the serum and blister fluids of BP patients; these cytokines may lead to a feedback loop and the perpetuation of the autoinflammatory process increasing MMP-9 secretion and CXCL10 expression from leukocyte cells [94].…”

“…Therefore, the marked association between OP and pemphigus can be explained at the molecular level by the immunopathogenic process and by the pro-inflammatory cells and cytokines shared in both disorders that lead to a net increase in bone resorption [ 92 ]. The guidelines for the treatment of BP of the British Association of Dermatologists recommend topical steroids as the first line of treatment if the pathology is limited; in the case of generalized pathology, the first line to be considered are topical steroids or high doses of systemic cortisone, while immunosuppressants (methotrexate, mycophenolate mofetil, azathioprine and dapsone) are to be considered the second line of treatment in extensive pathology, in patients who do not respond to oral corticosteroids or who have contraindications to it [ 92 ]. Considering that corticosteroid-induced osteoporosis is one of the most common and serious adverse effects in patients receiving long-term treatment, we observed, on the one hand, that in the study undertaken by Chovatiya et al [ 88 ], patients with PEM or BP as well as long-term systemic corticosteroid use had the highest odds of OP and fractures.…”

Long-term oral prednisolone exposure in primary care for bullous pemphigoid: population-based study

“…In this, 85.5% (448 of 524) of those with bullous pemphigoid diagnoses were prescribed an oral corticosteroid by their dermatologist at some point during their management. (22) We report that a more modest 69.6% of people are prescribed prednisolone in primary care, although this discrepancy may be largely attributed to the different settings. The higher proportion from the survey suggests that some people are exposed to prednisolone only in secondary, and not primary, care.…”

Validation study of bullous pemphigoid and pemphigus vulgaris recording in routinely collected electronic primary healthcare records in England

“…The benchmark diagnoses may be subject to misclassification bias due to a heterogeneous group of pemphigoid variants (eg, non-bullous pemphigoid, which presents with intense itching, a polymorphic rash, but no blisters), alternative approaches to making a diagnosis and differences in the diagnostic tests used to confirm the diagnosis. 22 23 Nevertheless, this was regarded as the best approach as external validation with hospital notes was not possible. Finally, validation was only possible for a third of patients and it may be that patients with a hospitalisation record for a blistering disease are not representative of the overall population of patients as they are more likely to have severe disease and associated comorbidities.…”