Successful Treatment of Brunsting-Perry Cicatricial Pemphigoid With Dupilumab

October 2021 | Volume 20 | Issue 10 | Case Reports | 1113 | Copyright © October 2021


Published online September 23, 2021

Haya S. Raef MSca, b, Sarina B. Elmariah MD, PhDb

aTufts University School of Medicine, Boston, MA
bDepartment of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA

Abstract
Brunsting-Perry is a rare variant of cicatricial pemphigoid, characterized by subepidermal bullae localized to the head and neck. Currently, treatment relies on non-specific immunosuppression, which in many cases, does not lead to a remission of treatment or significant clinical improvement. Dupilumab, a human monoclonal antibody against IL-4 receptor alpha, has been shown to provide relief of allergic inflammatory lesions and is the first biologic agent approved for the treatment of moderate-to-severe atopic dermatitis. We present the case of a 63-year-old patient with history of Brunsting-Perry cicatricial pemphigoid who proved refractory to multiple conventional therapies but was successfully treated with a dupilumab regimen of 300 mg every two weeks. This case suggests the potential role of dupilumab in the management of Brunsting-Perry cicatricial pemphigoid.

J Drugs Dermatol. 2021;20(10):1113-1115. doi:10.36849/JDD.6032

INTRODUCTION

Cicatricial pemphigoid (CP) is a chronic, subepidermal blistering disease of autoimmune origin.1 Brunsting-Perry cicatricial pemphigoid is a rare localized variant of CP characterized by a pruritic vesiculobullous eruption that predominately affects the head, neck, and scalp. In some cases, mild mucosal involvement may be present. Cutaneous lesions ultimately heal with atrophic scars, and thus control of disease progression and prevention of scarring is desirable.1 Currently, treatment relies on topical or systemic corticosteroids and non-steroidal immunosuppressive drugs.2 Dupilumab has been shown to provide relief of allergic inflammatory lesions and is the first biologic agent approved for the treatment of moderate-to-severe atopic dermatitis (AD).3 We present the case of a patient with history of Brunsting-Perry CP who proved refractory to multiple conventional therapies but responded well to treatment with dupilumab.

CASE

A 63-year-old female with a past medical history of cervical radiculopathy, obesity, allergic rhinitis and atopic dermatitis, presented to our clinic for evaluation of multiple pruritic and painful erosive scalp lesions. She reported occasional oral sores affecting the hard palate, buccal mucosa and gingiva, although had none at the time of her initial presentation. The patient had previously been diagnosed with Brunsting-Perry cicatricial pemphigoid (CP) by an outside dermatologist based on skin biopsy demonstrating a linear C3 band at the dermoepidermal junction on direct immunofluorescence. Serum autoantibodies against BP180 were mildly elevated and her ANA was positive. She had previously failed multiple topical and systemic treatments including potent and ultra-potent topical steroids, prednisone, mycophenolate, methotrexate, dapsone, doxycycline, nicotinamide, and rituximab 1000 mg IV infusions every 2 weeks.

Dermatological examination revealed scattered crusted erosions on the bilateral temporal and occipital regions of her scalp (Figure 1A). She had subtle eczematous patches and thin plaques on her upper arms and antecubital fossae as well widespread xerosis. Initial oral examination was unrevealing, however superficial erosions with tightly cuffed erythema were noted on the hard palate and gingiva upon subsequent physical examination.

A trial of methotrexate was initiated after her first visit, which resulted in improvement in eczematous lesions but not in