Vegetative lesions stem from a range of causes— typical examples include Pemphigus Vegetans (PV) and Pyoderma Vegetans (PDV). Clinicians face challenges in differential diagnosis, treatment, and long-term management. For pemphigus diseases, traditional first-line treatment involves systemic glucocorticoids, supplemented by second-line treatments such as non-specific immunosuppressants (e.g., cyclosporine). Dupilumab, a monoclonal antibody targeting IL-4Rα that works as an immunosuppressive biologic, has been reported in relevant studies for the treatment of PV. We report a 74-year-old female patient who presented with perianal nodules, plaques, and ulcers accompanied by pain for over 3 months, along with oral ulcers and weight loss. Skin biopsy revealed pseudoepitheliomatous hyperplasia and partial epidermal erosion; laboratory tests found positive pemphigus antibodies; skin secretion culture isolated Proteus mirabilis; ambulatory electrocardiogram indicated third-degree atrioventricular block; and PET-CT scan showed no tumor leisions. After treatment with Dupilumab combined with systemic glucocorticoids and Doxycycline, her skin lesions shrank and eventually healed. When encountering vegetative lesions with pseudoepitheliomatous hyperplasia and positive Dsg3, Pemphigus Vegetans should be considered first, but PDV or Paraneoplastic Syndrome still need to be ruled out. For patients with severe underlying diseases, dupilumab combined with low-dose glucocorticoids is a good option after weighing the benefits and risks of immunosuppressive therapy.
Wang et al. (Sat,) studied this question.