Bullous pemphigoid (BP) is a subepidermalautoimmune bullous disease, affecting mostly elderly patients. It is pathogenetically linked to autoantibodies directed against the 180 kD (BP180) and the 230 kD (BP230) antigens, two hemidesmosomal proteins promoting dermo-epidermal cohesion. BP typically presents with severe pruritus, preceding for several weeks the development of urticarial plaques and tense, mostly clear, blisters commonly on the flexural aspects of the limbs and the abdomen. Oral mucosal lesions are present in 10-20% of patients. Other clinical variants of BP are: prurigo-like BP, dyshidrosiform-like BP, erythroderma-like BP, ecthyma gangrenosum-like BP and localized (pretibial) BP. BP lesions in untreated patients may result in multiple erosions and crusts and pruritus may become torturous. Treatment of bullous pemphigoid is based on topical use of very potent steroids (clobetasol propionate), systemic steroids, dapsone, doxycycline, IVIG and more recently rituximab and immunoadsorption of pathogenic antibodies .

  • Specific challenges associated with the recognition of the condition

Mean delay in diagnosis of bullous pemphigoid is about 6 months. Main causes of delay are the lack of bullous lesions and the localization on one anatomic area. Almost 20% of patients present with non bulllous lesions, mimicking forms of eczema. This delay is associated with torturous pruritus and severe impact on patients’ quality of life.

  • Specific challenges associated with the diagnosis

Diagnosis of bullous pemphigoid is based on the combination of clinical, histopathological and immunological criteria. Atypical clinical variants, especially those with intense pruritus and non bullous lesions, should be investigated with immunofliuorescence techniques.

  • Specific challenges associated with the treatment

Although the use of super potent topical steroids are recommended as first line therapy (EADV /EDF Consensus), this therapeutic option has the disadvantages of poor practicality in bedridden patients, high rates of incompliance and poor accessibility in many countries.The main challenge in the treatment of BP remains the dose and duration of oral steroid treatment. Elderly patients, who are the majority of BP patients, suffer from steroid – induced side effects (uncontrolled diabetes, osteoporosis myopathy, cataract, glaucoma). New therapeutic options are needed. Drug induced BP remains also a challenge, in terms of recognition of the causative agent and of management.

  • Specific challenges associated with care of these patients over their lifespan – Quality of life issues – Gaps across the care continuum

Optimal management of bullous pemphigoid patients require a multidisciplinary approach by highly specialized dermatologists, internists, neurologists and general practitioners. Other specialists who can be involved in the management of corticosteroid side effects are rheumatologists, endocrinologists and ophtalmologists. Nurses are particularly involved during the acute phase of the disease to achieve disease control and epithelialisation of skin and especially when the topical application of high potency steroids is selected as monotherapy. Nursing staff plays also a major role in patients’ educational programs. Many patients complain of long-lasting symptoms or psychological troubles that frequently needs the intervention of psychologists.

Prevalence : unknown

 

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