Anesthetic Management of the Patient with Paraneoplastic Pemphigus: A case report / 대한마취과학회지

Paraneoplastic pemphigus is a distinct and rare autoimmune disease characterized by extensive and painful mucosal ulcerations and polymorphic desquamated skin lesions in the setting of an underlying neoplasm, typically of lymphoreticular origin. Thus difficulties in the management of anesthesia can be expected. A 66-years-old man was scheduled for removal of intraabdominal sarcoma associated with paraneoplastic pemphigus. Physical examination showed multiple erythematous bullae, crusts, plaques and target-like lesions on the whole body and desquamated erythematous skin lesions on the back and extremities. In the operating room, his right femoral artery was cannulated with a 20 G, 12.7 cm CVP catheter and left femoral and subclavian veins with 14 G, 20 cm CVP catheters, respectively. After application of 4 % lidocaine spray, his oropharynx and supraglottic area were evaluated under direct laryngoscopy and revealed multiple ulcerations on oral mucosa, but no distinct lesion on supraglottic area. Anesthesia was induced by rapid-sequence method with fentanyl, thiopental sodium and succinylcholine followed by endotracheal intubation. The endotracheal tube was held by a roll gauze around the neck and its cuff was minimally inflated to avoid overpressure against his tracheal wall. After the end of surgery, his oral cavity was suctioned with no remarkable bleeding, and tracheal wall including cuff-contacted area was evaluated under fiberoptic bronchoscopy, revealed intact wall without any bulla or ulceration. The patient was transferred to intensive care unit for proper postoperative management after extubation of endotracheal tube.

Immunofluorescent and Immunoblotting Features of Paraneoplastic Pemphigus / 中华皮肤科杂志

Objective To investigate the immunofluoresce nt and immunoblotting features of paraneoplastic pemphigus(PNP).Methods Sera were tested by indirect immunofluorecence(IIF)and immunoblotting(IB)in four patients with PNP.Results Binding of IgGand C3to the intercell ular spaces of epidermis was found in IIF with rat bladder as substrate.The titers decreased after the excision of tumo rs.The autoantibodies also bound to sub-strates of the epithelia of human ski n,rat tongue and esophagus in those p atients.The patients' sera recogni zed 210kD and 190kD antigens from human kera tinocyte extracts with IB analysis.Conclusions IIF with rat bladder as substrate can be used a screening test for PNP.PNP tends to involve epithelia of mucosa.The results of IB test m ay confirm the diagnosis of PNP of the 4p atients.[

Clinical features and surgical treatment of retroperitoneal Castleman′s disease with paraneoplastic pemphigus / 中国普通外科杂志

Objective To investigate the clinical characteristics and surgical treatment of retroperitoneal Castleman′s disease (CD) with paraneoplastic pemphigus (PNP).Methods The clinical symptoms, laboratory, histopathologic, CT findings and results of surgery in 7 patients were evaluated.Results All of 7 patients presented with PNP.Retroperitoneal tumors were found by CT examination and surgically removed.Castleman disease was confirmed by histopathology.Reoperation was performed to resect the recurrent tumor in one patient.The signs of PNP were dissolved completely or partially after operation.Conclusion Retroperitoneum CD with PNP are characteristic of distinctive clinical features.Surgical excision improves the mucocutanous manifestation of PNP.