BACKGROUND: Paraneoplastic pemphigus (PNP) is an autoimmune-related acquired bullous disease. Delayed diagnosis and treatment of this clinically rare disease often result in poor prognosis. METHODS: Between January 1999 and December 2009, 22 patients with confirmed PNP who underwent surgical resection of underlying tumors were enrolled in this study. Clinicopathologic characteristics, treatment options, and perioperative and long-term results were analyzed. RESULTS: Among 22 patients, 2 patients died of severe infection several weeks after surgery. Postoperative major complications included pulmonary infections (n = 10) and septicemia (n = 4). Respiratory symptoms persisted in 13 patients. Tumors were completely resected in 20 patients. Two patients whose tumors were not completely resected died of relapse 2 and 32 months after surgery. Two patients with completely resected tumors died of respiratory failure 10 and 24 months after surgery, respectively. One patient whose pathological result was follicular dentritic cell sarcoma had a relapse recently. The remaining 15 patients have survived till now. CONCLUSIONS: Early detection, prompt treatment, and complete resection of PNP can effectively decrease the mortality and speed up the recovery.
BACKGROUND:Paraneoplastic pemphigus (PNP) is an autoimmune-related acquired bullous disease. Delayed diagnosis and treatment of this clinically rare disease often result in poor prognosis. METHODS: Between January 1999 and December 2009, 22 patients with confirmed PNP who underwent surgical resection of underlying tumors were enrolled in this study. Clinicopathologic characteristics, treatment options, and perioperative and long-term results were analyzed. RESULTS: Among 22 patients, 2 patients died of severe infection several weeks after surgery. Postoperative major complications included pulmonary infections (n = 10) and septicemia (n = 4). Respiratory symptoms persisted in 13 patients. Tumors were completely resected in 20 patients. Two patients whose tumors were not completely resected died of relapse 2 and 32 months after surgery. Two patients with completely resected tumors died of respiratory failure 10 and 24 months after surgery, respectively. One patient whose pathological result was follicular dentritic cell sarcoma had a relapse recently. The remaining 15 patients have survived till now. CONCLUSIONS: Early detection, prompt treatment, and complete resection of PNP can effectively decrease the mortality and speed up the recovery.
Authors: G J Anhalt; S C Kim; J R Stanley; N J Korman; D A Jabs; M Kory; H Izumi; H Ratrie; D Mutasim; L Ariss-Abdo Journal: N Engl J Med Date: 1990-12-20 Impact factor: 91.245
Authors: Frits van Rhee; Eric Oksenhendler; Gordan Srkalovic; Peter Voorhees; Megan Lim; Angela Dispenzieri; Makoto Ide; Sophia Parente; Stephen Schey; Matthew Streetly; Raymond Wong; David Wu; Ivan Maillard; Joshua Brandstadter; Nikhil Munshi; Wilbur Bowne; Kojo S Elenitoba-Johnson; Alexander Fössa; Mary Jo Lechowicz; Shanmuganathan Chandrakasan; Sheila K Pierson; Amy Greenway; Sunita Nasta; Kazuyuki Yoshizaki; Razelle Kurzrock; Thomas S Uldrick; Corey Casper; Amy Chadburn; David C Fajgenbaum Journal: Blood Adv Date: 2020-12-08