Understanding bullous pemphigoid: a rare skin condition

Bullous pemphigoid is a rare skin condition that predominantly occurs in elderly people. Although corticosteroids are still the standard treatment option for patients with this disease, novel options give hope to this population. Prof. Barbara Horváth (University Medical Center 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,” Prof. Horváth stated at the start of her presentation [1]. 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 Prof. Horváth. Patients with bullous pemphigoid are usually fragile, with multiple comorbidities and polypharmacy. The 1-year and 3-year mortality rates are 22–38% and 40– 46%, respectively [2]. 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].

Challenges in managing bullous pemphigoid

“There are several challenges in treating patients with bullous pemphigoid,” said Prof. Horváth [1]. 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,” added Prof. Horváth. 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 cause this condition.

Current treatment protocols

Subsequently, Prof. Horváth shared the therapeutic algorithm used in the patient population (see Figure) [4]. In general, systemic corticosteroids are the first-line choice, with a typical starting dose of 0.5 mg/kg/day of prednisone. If no response is seen 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.

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 Prof. Horváth [1]. “In younger patients or patients with multiple sclerosis, rituximab may be an effective option. However, the evidence for these options is limited,” emphasised Prof. Horváth. Dapson, doxycycline, mycophenolate mofetil, azathioprine, immunoglobulin, and cyclophosphamide are potential second-line and third-line options.

Figure: Therapeutic algorithm for bullous pemphigoid [4]



IV, intravenous; IVIG, intravenous immunoglobulin.

“Another important aspect in managing bullous pemphigoid is wound care,” Prof. Horváth continued. Prof. Horváth 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.

Finally, in the case of therapy-induced bullous pemphigoid, the causative therapy must (temporarily) be 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 Prof. Horváth.

Promising new treatments in development

“Although the treatment algorithm has not changed much in the last years, there is hope for patients with bullous pemphigoid,” said Prof. Horváth. Currently, rituximab, omalizumab, and dupilumab are 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 Prof. Horváth. “It is, however, challenging to include these fragile patients with a high mortality rate in a clinical trial.”

1. Horváth B. Therapeutische strategie bij bulleus pemfigoid. Dermatologendagen 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. Lamberts A, et al. NTvDV. 2024; 34(3):4-7.

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Posted on 31 May 20244 June 2024