Paraneoplastic pemphigus
Overview
The least common but most severe form of pemphigus disease resulting in painful blisters on the mouth, lips, skin, and esophagus. Typically associated with cancer.
Common Symptoms
Oral blisters, skin lesions, itchiness, as well as respiratory and/or gastrointestinal issues.
Coexisting Diseases and Conditions
B-cell lymphoma, chronic lymphocytic leukemia, Castleman’s disease, Waldenstrom’s macroglobulinemia, thymoma, and myasthenia gravis.
Risk Factors and Prevalence
Age between 45 and 70 years. Children with Castleman’s disease, a disease caused by enlarged lymph nodes, are commonly affected by this condition. At this time, there are no known correlations between the disease’s incidence and any sex, race, or place of origin.
Recent Research
- Paraneoplastic pemphigus associated with Castleman disease: A muilticenter case series (2022)
- Paraneoplastic pemphigus: A detailed case series from Netherlands revealing atypical cases (2022)
- Clinical and pathological features of bronchiolitis obliterans requiring lung transplantation in paraneoplastic pemphigus associated with Castleman disease (2022)
- Paraneoplastic pemphigus: Revised diagnostic criteria based on literature analysis (2021)
Sources
- Article Sources
Goldberg, L. J., MD. (2021, March 26). Paraneoplastic Pemphigus: Background, Pathophysiology, Etiology.
Paraneoplastic pemphigus: a short review. (2016). PubMed Central (PMC). https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5042195/#:%7E:text=Paraneoplastic%20pemphigus%20(PNP)%20is%20a,atypical%20pemphigus%20with%20associated%20neoplasia
Paraneoplastic pemphigus | DermNet NZ. (n.d.). Retrieved June 12, 2021, from https://dermnetnz.org/topics/paraneoplastic-pemphigus/