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Case Discussion

Case Discussion. Paraneoplastic Pemphigus Presented by Ri 吳曉婷 , 黃啟倫. Basic Data. 陳 XX, 44 y/o married women DM (-), HTN(-) Denied other systemic disease Denied any allergic history Denied previous HSV infection.

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Case Discussion

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  1. Case Discussion Paraneoplastic Pemphigus Presented by Ri 吳曉婷,黃啟倫

  2. Basic Data 陳XX, 44 y/o married women DM (-), HTN(-) Denied other systemic disease Denied any allergic history Denied previous HSV infection

  3. Brief History and Admission Course (I) • The 44 y/o female patient visited our ER on 2002/04/01 due to refractory diffuse oral and pharyngeal ulcer for about 8 days. • Besides, she had productive cough for about 1 month and conjunctivitis (LMD & herb medicine) • Under the impression of Steven-Johnson syndrome related to HSV infection or drug, she was admitted to 15A ward for further care.

  4. Brief History and Admission Course (II) • Physical examination General appearance: ill-looking Consciousness: clear Conjunctiva:injected Sclera: anicteric Oral mucosa:diffuse erosion with yellowish crust over the whole buccal and lingual mucosal surface

  5. Brief History and Admission Course (III) • 4/2 : admission and start IV steroid treatment; skin biopsy was done • 4/8: Skin biopsy result (lower lip) : suprabasilar blister with sloughed epidermis, pemphigus was likely Fever was noted─ suspect UTI related Empiric antibiotics were used • 4/13 : DC steroid due to poor infection control

  6. Brief History and Admission Course (IV) • 4/14 : Skin biopsy (Back) : suprabasilar acantholysis with blister formation Direct immunofluoresence stain : linear C3 deposition at basement membrane zone IgG at intercellular space of epithelium check tumor marker: CA125(202.64)↑ Highly suspect paraneoplastic pemphigus • 4/15 : Abd. sonography : splenomegaly with hypoechoic lesion, susp. tumor infiltration (Lymphoma) No pelvic mass was noted

  7. Brief History and Admission Course (V) • 4/16 : Abd. CT : 1.Huge hypodense splenic tumor of 13x11x18 cm 2.Hypodense mass at the pelvis with ascites 3.1.2 cm hypodense lesion at liver Imp: 1.Uterine tumor with spleen and liver metastasis 2.Non-Hodgkin’s lymphoma

  8. Brief History and Admission Course (VI) Biopsy of the splenic tumor was unfeasible due to high hemorrhagic risk→ diagnostic laparoscopic splenectomy was suggested Solumedrol 40mg q12h iv was used to control her pemphigus activity • 4/18 : consult infection specialist for poor control of infection intraperitoneal TB was suspected • 4/22 : fever subsided after four combined anti-TB therapy (Isoniazid, Rifampin, Pyrazinamide and Ethambutal). • 4/23 : scheduled for op but was postponed due to difficult intubation risk • 5/13 : operation

  9. During Anesthesia • Patient’s condition: WBC Hb PLT BP 9570 8.9 149K 65/110 • ETGA • Laryngoscopic intubation, with 7.0 mm tube fixed at 20cm, checked by fibroscope • Agents : Rubinol, pentothol, Fentanyl, Atropin, S.C.C, Atracurium, Enlon • Monitoring : CVP, pulse oxymetry, A-line, pressure cuff, ECG pad • Cuff pressure < 20 cmH2O; Airway pressure < 15 cm H2O • Tidal volume < 10c.c./kg • No vaseline gel was used over face

  10. Extubtion • Performed with fibroscope, no new visecles or bullae noted in the airway • Could breath spontaneously • No airway obstruction • No hemoptysis • No skin lesion at the site of IV cannulation and BP cuff placement • No lesion at pressure point • P’t was transferred to ICU

  11. Post-operative Course • Profound new vesicles were found when she was transferred back to the ward and the Solu-medrol was increased to 240mg per day. New vesicles stopped in 5 days and old ones healed almost completely. Solu-medrol was tapered to 80mg per day after new vesicles stopped formation. • Pathology of the splenic tumor revealed mixed small cleaved and large B-cell lymphoma • BM aspiration : (+) • BM biopsy : (-)

  12. Post-operative Course • 5/22 : Epigastralgia WBC : 15990, Amylase : 1171, Lipase: 3695 Acute pancreatitis was likely Abd. CT showed acute pancreatitis with hemorrhage complication • 5/27 : Transfer to 13B ward for further care • 5/30 : Acute pancreatitis was controlled (Amylase :218; Lipase : 428; LDH: 1017) She was transferred to 12D ward for chemotherapy

  13. Bullous Disease • Pemphigus Vulgaris • Pemphigus Foliaceus • Paraneoplastic Pemphigus • Bullous Pemphioid • Cicatricial Pemphigoid • Pemphigoid Gastationis • Dermatitis Herpitifomis • Linear IgA Bullous Dermatitis • Epidermolysis Bullosa Acquisita

  14. Pemphigus and Pemphigoid Disease

  15. Paraneoplastic Pemphigus • Autoimmune disease that accompanies an overt or occult neoplasm and cause blisters • The most commonly associated neoplasms: -- Non-Hodgkin’s lymphoma -- Chronic lymphocytic leukemia -- Castleman’s disease -- Thymoma -- Retroperitoneal scarcoma -- Waldenstrom’s macroglobulinemia International Journal of Dermatology 2001,40:367-72 Lancet 1999, 354:667-72

  16. Clinical Manifestations • Mucosal: intractable stomatitis causing erosions and ulcerations in the oral mucosa • Cutaneous: trunk and proximal extremities flaccid or tense blisters with or without erosions • Respiratory: obstructive ventilatory defect inflammation, vesiculation of tracheobronchial epi.

  17. Airway Involvement

  18. Immunologic Studies • Immunoprecipitation studies: gold standard for the diagnosis of paraneoplastic pemphigus • Respiratory and urinary bladder epi: desmoplakin(+), desmoglein(-)  for distinguishing paraneoplastic pemphigus from other forms of pemphigus

  19. Pathogenesis • Combination of humoral and cell-mediated autoimmunity • Neoplasm of immune origin may cause dysregulation of immune system • Antigenic components that cross-react with epidermal cell surface proteins

  20. Diagnosis

  21. Respiratory Failure in Paraneoplastic Pemphigus • Bronchiolitis obliterans • Infection, toxic effects induced by chemotherapy, neoplasia and autoantibody-mediated pulmonary injury • Acantholysis with deposits of IgG in the bronchial epithelium. • Antiplakin antibodies NEJM 1999; 340(18): 1406-10, 1999

  22. Case 1 • 46 y/o female • Abdominal hysterctomy and bilateral salphingo-oophorectomy • Pemphigus vulgaris for 8 years • Prednisone 10mg + azathioprine 100mg • PE: painful bullous formations in the mouth and oropharynx Regional anaesthesia in pemphigus vulgaris. Anaesthesia, 1992; 47:74

  23. Process and Results • Spinal anesthesia • Hydrocortisone 100mg iv. preoperative and during surgery • 25-gauge spinal needle inserted at the L2-3 • No lesions at the site of spinal needle puncture, blood pressure cuff, intravenous cannulation, or oxygen mask application (the face has been pretreated with vaseline gel)

  24. Case 2 • 45 y/o man • Exploratory laparotomy for suspected PPU • Pemphigus vulgaris with bullae over face, trunk, extremities, oral cavity and pharynx • Prednisolone 60mg/day + NSAID Anaesthetic management of a patient with pemphigus vulgaris for emergency laparotomy. Anaesthesia 2000; 55:155-162

  25. Process and Results • General anesthesia • Monitors: CVP, A-line, pulse oximetry, end-tital gas monitoring • Facial lesions were covered with 1% hydrocortisone cream and soft cotton sponges before placing the facemask • Pre-OP laryngoscope: bleeding from mucosal lesion of oropharynx  throat pack soaked with saline adrenaline • Induction: thiopental + succinylcholine • Maintenance: NO 66% + halothane 0.5% in oxygen • Extubation under deep halothane anesthesia • Hydrocortisone pre-operative and post-operative

  26. Case 3 • 49 y/o female, Stage IV vulvar carcinoma • Radical vulvectomy and bil. femoral and pelvic node disection • Bullous pemphigoid diagnosed 2 months before • Admitted for iv steroid administration and hydration before surgery due to poor control • Prednisone 80mg + azathioprine 50mg bid • PE: ulcerated lesion on the lower gums, no intraoral lesions Anesthetic management of a patient with bullous pemphigoid. Anesth Analg 1989; 69:537-40

  27. Process and Results • Spinal anesthesia • Premedicated with morphine, scopolamine and hydrocortisone • Adhesive tape, the IV catheter, precordial stethoscope, ECG pads and blood pressure cuff as usual • New bullous and erythematous lesions of the face and inframammary area • No lesions at the site of spinal needle puncture, blood pressure cuff placement, or IV cannulation • No lesions at pressure points

  28. Discussion • New bullae formation • Infection and dehydration • Side effects of medication • Choice of anesthesia • Endotracheal intubation

  29. New Bullae Formation • Prevention of : -- friction and trauma -- local anesthesia infiltration -- spirit swab -- adhesive tape -- ECG electrodes and blood pressure cuff

  30. Infection and Dehydration • Fluid replacement for dehydration • Correct electrolyte abnormalies • The choice of injection site should be away from skin lesion

  31. Side Effects of Medication • Steroid -- sodium and fluid retention, hypokalemic alkalosis, peptic ulcer, hyperglycemia, impaired wound healing • Azathioprine -- reversible leukopenia and thrombocytopenia, secondary to bone-marrow supression, and hepatotoxicity with biliary stasis

  32. Choice of Anesthesia • Controversial • Ketamine • 131 patients, ETGA -- no intraoperative or postoperative airway obstruction -- 6 instances of facial and intraoral bullae formation

  33. Endotracheal Intubation • ET tube should be liberally lubricated • Face and lips should be pretreated with 1%hydrocortisone cream and vaseline gel • Maintaining minimal cuff inflation pressure • Macintosh rather than a Miller laryngoscope blade • Smaller tube • ET tube should be secured with a soft cloth bandage • Postoperative laryngoscope?

  34. In Our Patient • Potential of more generalized involvement • Avoid of adhesive tape • Avoid of ECG electrodes and blood pressure cuff if possible • Pretreatment of face and lip • Smaller ET tube

  35. Thanks For Your Attention

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