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We report the case of a 3-year-old boy who was diagnosed with childhood pemphigus vulgaris having developed oral lesions, gastrointestinal symptoms with esophageal involvement, and failure to thrive. He had a markedly increased total serum IgE level and peripheral blood eosinophilia. The pemphigus was recalcitrant to conventional therapies and, based on the coexisting characteristics of Th2 immune deviation, he was treated with dupilumab and has had sustained clinical improvement since starting treatment.

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Article Synopsis
  • Pemphigus foliaceus (PF) and bullous pemphigoid (BP) are two different autoimmune skin diseases caused by autoantibodies affecting skin adhesion proteins.
  • The occurrence of both PF and BP in a single patient is uncommon, as demonstrated in the case of a 72-year-old male who exhibited symptoms of both conditions.
  • Treatment for this patient included immunoglobulin, intravenous dexamethasone, oral triamcinolone, and minocycline, leading to significant improvement and emphasizing the need for precise diagnosis and effective treatment.
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Behçet's disease (BD) is a systemic vasculitis characterized by recurrent painful oral and genital ulcers, uveitis, and skin lesions. Pemphigus vulgaris (PV), on the other hand, is an autoimmune blistering disorder affecting the mucous membranes and skin, characterized by the presence of intraepidermal vesicles. Herein, we present a female in her 40s with a history of BD who presented to the emergency department with worsening oral and vaginal ulcers and extensive bullae of four months onset.

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Background: Pemphigus is a group of rare but serious autoimmune blistering disorders, affecting skin and mucus membrane. Different reports have been published in respect to the coexistence of pemphigus with neoplasms, especially lympho-proliferative ones.

Case: Here, we have reported a patient previously diagnosed with pemphigus vulgaris (PV) who developed esophageal squamous cell carcinoma (SCC).

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