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  • Presentation

F024-Pemphigus and Pemphigoid: Evidence Based Updates

Description

The presentation covers multiple cases of patients with pemphigus and pemphigoid, highlighting diagnosis challenges and treatment responses. Case 1 involves a 60-year-old male with persistent hoarseness and painful oral lesions; initially misdiagnosed, he was later treated for pemphigus with prednisone and rituximab, leading to significant improvement. Case 2 features a 35-year-old female with known pemphigus experiencing hyper salivation, later managed effectively with increased immunosuppressive therapy. Case 3 describes a 45-year-old male with oral sores, who, despite aggressive treatment, had a poor outcome due to complications from cryptococcal meningitis. Another patient had a severe flare post-noncompliance with medications, ultimately diagnosed with squamous cell carcinoma. Additionally, an 87-year-old man with a generalized rash and negative classic indices was diagnosed with a rare phenotype of pemphigoid after extensive testing, emphasizing how multifaceted and sometimes delayed the diagnosis and treatment of these autoimmune conditions can be. The cases demonstrate the importance of continuous monitoring, early intervention, and adaptability in treatment to manage pemphigus and pemphigoid effectively.

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Conclusions

  • Early recognition and appropriate management of symptoms in pemphigus patients can lead to positive treatment outcomes.
  • Conversely, misdiagnosis or delayed treatment can result in severe complications or mortality in pemphigus patients.
  • Immunosuppressive therapies and chemotherapy may increase the risk of infections and complications such as cryptococcal meningitis.
  • It is crucial to monitor for signs of disease flare in patients with a history of pemphigus, as subtle changes can indicate worsening conditions.
  • Communication and observational skills are essential in diagnosing and managing non-verbal patients with pemphigus.
  • Treatment should adjust based on patient response and emerging symptoms, including hypersalivation or skin eruptions.
  • High suspicion is necessary for drug-induced pemphigoid in patients undergoing immune checkpoint inhibitor therapy, as timing of onset can vary significantly.
  • Cases of bullous pemphigoid associated with immune checkpoint therapy might show better responses in tumor treatment than those without such reactions.
  • Association of Bullous Pemphigoid With Immune Checkpoint Inhibitor Therapy in Patients With Cancer: A Systematic Review, Maria S. Asdourian, MPhil1,2; Nishi Shah, BS2,3; Ted V. Jacoby, BS2,4; et al
  • Nonbullous pemphigoid: Insights in clinical and diagnostic findings, treatment responses, and prognosis, Aniek Lamberts, MD, Joost M. Meijer, MD, PhD, Hendri H. Pas, PhD, Gilles F. H. Diercks, MD, PhD, Barbara Horváth, MD, PhD, Marcel F. Jonkman, MD, PhD
  • Lichenoid dermatitis, spong. dermatitis, vitiligo have been associated with improved tumor response and survival outcomes after PD-1 and PDL-1 inhibitors.