Therapeutic strategies for bullous pemphigoid

Bullous pemphigoid is a rare skin condition that predominantly occurs in elderly patients. Although corticosteroids are still the standard treatment option for patients with this disease, novel options give hope to this population. Dr Barbara Horvath (University Medical Centre Groningen, the Netherlands) presented an update on disease management and emerging treatment options.

“Despite our understanding of the pathophysiology of bullous pemphigoid, there is still no approved treatment for this disease,” Dr Horvath stated at the start of her presentation. According to Dr Horvath, bullous pemphigoid is a rare autoimmune skin disease that mostly occurs in individuals over 70 years of age. It is characterised by intense itching, urticarial plaques, and large blisters. “In the last decade, the prevalence has increased with a factor of 2 to 5 due to the ageing population,” added Dr Horvath. Patients with bullous pemphigoid are usually fragile, with multiple comorbidities and polypharmacy. The 1- and 3-year mortality rates are 22-38% and 40-46%, respectively [2], being considered high. Furthermore, bullous pemphigoid is associated with neurological conditions such as Parkinson’s disease, epilepsy, and dementia, and with psychiatric diseases like schizophrenia, depression, and bipolar disorder [3].

“There are several challenges in treating patients with bullous pemphigoid,” said Dr Horvath. First, visiting a dermatologist is often difficult for these fragile patients. “We often have to rely on telephone appointments to serve patients with bullous pemphigoid.” Secondly, the comorbidities and polypharmacy of many patients urge dermatologists to adjust pharmaceutical interventions regularly. “We cannot always use systemic corticosteroids and have to use more local treatments in some cases,” according to Dr Horvath. Furthermore, bullous pemphigoid is frequently induced by pharmaceutical therapies. Diuretics, anticholinergics, immune checkpoint inhibitors, and dipeptidyl peptidase-4 inhibitors are examples of drugs that can induce bullous pemphigoid.

Hereafter, Dr Horvath showed the therapeutic algorithm used in the patient population [4]. In general, corticosteroids are the first-line choice, with a typical starting dose of 0.5 mg/kg/day of prednisone. If there is no response in 2-3 weeks, the dose may be raised to 0.75 mg/kg/day. Tapering can be performed after disease control is achieved, usually after 6 months [4]. Another first-line option is topical clobetasol 20-30 g/day, given for 4 months. “Low-dose methotrexate (5-15 mg/week) is an alternative option in older patients with bullous pemphigoid,” added Dr Horvath. “In younger patients or patients with multiple sclerosis, rituximab may be an effective option. However, the evidence for these options is limited,” emphasised Dr Horvath. Dapson, doxycycline, mycophenolate mofetil, azathioprine, immunoglobulin, and cyclophosphamide are potential second- and third-line options.

“Another important aspect in the management of patients with bullous pemphigoid is wound care,” Dr Horvath continued. Dr Horvarth suggested administering painkillers during wound care, using non-adhesive bandages, and leaving blisters mostly closed to avoid secondary skin infections. Local antibiotics or antiseptics may be used in case of impetiginisation.

“Although the treatment algorithm hasn’t changed much in the last years, there is hope for patients with bullous pemphigoid,” said Dr Horvath. Currently, rituximab, omalizumab, and dupilumab are agents being tested in clinical trials or for which evidence is collected through case series. “Given the knowledge we have on the pathophysiological mechanisms that drive the disease, there are more potential targets that could be investigated,” argued Dr Horvath. “It is, however, very difficult to include these fragile patients with a high mortality rate in a clinical trial.”

Finally, in the case of therapy-induced bullous pemphigoid, the causative therapy must be (temporarily) stopped. Most dermatologists use the standard treatment algorithm to treat these patients. “In case of immunotherapy-induced bullous pemphigoid, high-dose corticosteroids are needed, and the inducing therapy is often discontinued permanently,” said Dr Horvath.

1. Horvath B. Therapeutische strategie bij bulleus pemfigoid. DDD 2024, 11-12 April, Amsterdam, the Netherlands.
2. Kridin K, et al. Front Med (Lausanne). 2018;5:220
3. Kridin K, et al. Immunol Res. 2018;66:255-270
4. Borradori L, et al. JEADV. 2022;36:1689-1704

Medical writing support was provided by Robert van den Heuvel.
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Posted on 17 April, 2024