Abstract
Pemphigus defines a group of rare autoimmune blistering diseases that affect the skin and mucous membranes, with pemphigus vulgaris being the most common form that has increased morbidity and mortality in the absence of an early diagnosis and treatment.We report the case of a 24-year-old male with an atypical form of pemphigus vulgaris with cutaneous onset and subsequent involvement of the oral cavity. The management of the patient initially consisted of long-term systemic corticosteroid therapy. Following a mild form of SARS-CoV-2 infection and a flare-up of the disease in this context, which was not controlled with high doses of systemic corticosteroids, targeted therapy with rituximab was initiated but immediately stopped due to the manifestations of urticaria and angioedema. Considering the magnitude of these reactions, dapsone systemic therapy i.e., a steroid-sparing agent with minimal risk of infections, was started and managed to control the underlying disease.The management of this case of pemphigus vulgaris was challenging for both the patient and his physician, as the patient developed COVID-19 which caused disease complications and implied additional costs. This case highlights the importance of an accurate diagnosis given the atypical onset of the disease and the financial limitations with the impossibility of performing all confirmatory diagnostic tests.
Highlights
Pemphigus defines a group of rare chronic autoimmune diseases that affect the skin and mucous membranes with severe impairment of the quality of life
We report the case of a 24-year-old male with an atypical form of pemphigus vulgaris with cutaneous onset and subsequent involvement of the oral cavity
At two months post-SARS-CoV-2 infection, in the absence of therapeutic response to high doses of corticosteroids, we considered initiating therapy with rituximab
Summary
Pemphigus (derived from the Greek term ‘pemphix’, which means blister) defines a group of rare chronic autoimmune diseases that affect the skin and mucous membranes with severe impairment of the quality of life. 50% to 70% of the patients with pemphigus vulgaris initially present with mucosal lesions, oral lesions, with subsequent involvement of the skin [5]. At the time of COVID-19 diagnosis, the patient was still on systemic corticosteroids for his underlying disease (the dose was slowly tapered reaching half of the initial dose decided at the first hospitalization). Considering the severe allergic response, after the allergy assessment and the clinical stabilization of the patient, we decided to stop the therapy with rituximab and to continue systemic corticosteroids in combination with dapsone. The patient had received the combination of dapsone and low doses of systemic corticosteroids for five months, with the subsequent complete elimination of corticosteroids from the treatment regimen, with no signs of disease reactivation
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