Zach Ney
Intravenous Immunoglobulin as a Potential Treatment for Refractory Lichen Planus Pemphigoides- Two Cases
Authors: Lauren Madigan, MD
Department: Dermatology
Background:
Lichen planus pemphigoides (LPP) is a rare, immune-mediated blistering disease with characteristics of lichen planus (LP) and bullous pemphigoid (BP). LPP can be idiopathic or may be associated with chronic viral infections or medications, including immune checkpoint inhibitors. Treatment varies but generally involves topical and systemic corticosteroids, antimalarials, dapsone, acitretin, and other steroid-sparing therapies. We report two cases of treatment-refractory LPP secondary to immunotherapy which were successfully treated with intravenous immunoglobulin (IVIg).
Methods/Case Introductions:
Case 1: A woman in her 60s with history of malignant melanoma presents with rash and mucositis shortly after starting pembrolizumab. After determining a diagnosis, patient underwent treatment over the next 18 months with multiple therapies including dapsone, methotrexate and more, all without resolution.
Case 2: A man in his 70s with a history of gastric adenocarcinoma presents for widespread rash and oral ulcers 9 months after starting pembrolizumab. Over the following months, he would trial many different topical and systemic treatments without any significant improvement.
Results:
In both cases presented here of LPP due to immune checkpoint inhibitors which were refractory to traditional treatments, patient’s condition improved significantly after the initiation of IVIG. Symptoms improved rapidly after starting IVIG, particularly pain, and even oral and cutaneous lesions improved after several months of treatment.
Conclusions:
IVIg should be considered as a potentially safe, effective, and lasting treatment in patients with immune checkpoint inhibitor-induced LPP. These two cases highlight its efficacy and show the potential for relatively rapid and complete resolution of symptoms related to LPP.
