|Year : 2007 | Volume
| Issue : 6 | Page : 525-527
Anaesthetic management for paraneoplastic pemphigus
ST Dave1, V Chandrashekhar2, SK Kamath3, LV Dewoolkar4
1 MD, DNB, Associate Professor, Department of Anaesthesia, Seth G S Medical College & K.E.M Hospital, Parel, Mumbai 12, India
2 MD, FCPS, Ex- Lecturer, Department of Anaesthesia, Seth G S Medical College & K.E.M Hospital, Parel, Mumbai 12, India
3 MD, DA, Ex- Professor, Department of Anaesthesia, Seth G S Medical College & K.E.M Hospital, Parel, Mumbai 12, India
4 Head of Department, Department of Anaesthesia, Seth G S Medical College & K.E.M Hospital, Parel, Mumbai 12, India
|Date of Acceptance||20-Sep-2007|
|Date of Web Publication||20-Mar-2010|
S T Dave
4/C, BINDIYA, Reclamation area, Bandra (West), Mumbai - 400050
Source of Support: None, Conflict of Interest: None
The article sums up the anaesthetic management of a patient with paraneoplastic pemphigus, undergoing excision of a retroperitoneal tumor. General anaesthesia with tracheal tube was administered, with perioperative steroid cover.All precautions to prevent exacerbation of pemphigus were taken.
Keywords: Complications, Pemphigus vulgaris, Paraneoplastic pemphigus Anaesthesia: General, Intubation
|How to cite this article:|
Dave S T, Chandrashekhar V, Kamath S K, Dewoolkar L V. Anaesthetic management for paraneoplastic pemphigus. Indian J Anaesth 2007;51:525-7
|How to cite this URL:|
Dave S T, Chandrashekhar V, Kamath S K, Dewoolkar L V. Anaesthetic management for paraneoplastic pemphigus. Indian J Anaesth [serial online] 2007 [cited 2020 Oct 21];51:525-7. Available from: https://www.ijaweb.org/text.asp?2007/51/6/525/61192
| Introduction|| |
Pemphigus comprises a group of chronic bullous diseases originally named by Wichmann in 1791. The disease is characterized by blisters and erosions of the skin and the mucus membranes. Involvement of the oropharynx is present in about 50% of the patients with pemphigus  . The facial, oropharyngeal and laryngeal lesions produce difficulties in airway management. Cautious gentle airway instrumentation is imperative. Paraneoplastic pemphigus is a new term for neoplasm induced pemphigus. Castleman's tumor is disproportionately associated with paraneoplastic pemphigus. Extra care to protect skin and mucus membranes from trauma during procedures is essential. Adrenocortical suppression following prolonged steroid therapy is another hazard to be tackled in the perioperative period  .
We report here the anaesthetic management of a patient with paraneoplastic pemphigus with oral lesions and a provisional diagnosis of phaeochromocytoma ,under long term steroid coverage, undergoing excision of a retroperitoneal tumor, requiring general anaesthesia with tracheal tube.
| Case report|| |
A 25-year-old woman with a history of multiple oral ulcers and raised red lesions over chest, abdomen and fingers for 18 months was attending the dermatology outpatient department of our hospital. Diagnosis of paraneoplastic pemphigus was made histologically from the biopsy of a hand lesion. The patient was put on steroid therapy ( prednisolone 20mg o.d.) . She was referred to the endocrine department for hypertension and diffuse goiter. Her blood pressure was controlled with doxazosin 2 mg b.d. and atenolol 50mg o.d. Thyroid functions were within normal limits. Computerised axial tomography of the abdomen revealed a tumor of 7 x 4 x 7 cm in the right adrenal fossa encircling the inferior vena cava.
Preoperative airway assessment revealed mouth opening of only 2 cm and Mallampatti classification grade III as she was unable to protrude the tongue fully because of painful, scarred oral lesions.
She also had pemphigus lesions over hand, chest and abdomen. As there was no hoarseness of voice , laryngeal pemphigus was not suspected. Associated diseases such as rheumatoid arthritis , myasthenia gravis , collagen diseases and pernicious anaemia were ruled out through investigations.
In view of the slight increase in the 24 hour urinary metanephrine levels and the site of tumor ( right adrenal fossa),aprovisional diagnosis of right haeochromocytoma was made.
Routine investigations including blood sugar , serum electrolytes and serum proteins were within normal limits. Pulmonary function tests could not be performed since the oral ulcers bled on touch. Serum cortisol levels were low due to prolonged exogenous steroid therapy.
No intramuscular premedications were given . Only a mild sedative was given orally the night prior. Antihypertensive medications were given orally preoperatively. Preoperatively vital parameters were within normal limits. In anticipation of intraoperative bleeding (the tumor was vascular and encircling the inferior vena cava) the left radial artery, the basilic and the two peripheral veins were cannulated.
With utmost care the cannulae were secured in place by suture and gauze bandaging. Hydrocortisone 200mg and glycopyrrolate 0.2 mg intravenously were given before the induction of anaesthesia.
Anaesthesia was induced with thiopentone 250mg and succinylcholine75mg. Midazolam 2mg and fentanyl 100mcg were given for sedation. Ventilation was provided by applying minimal pressure on the face mask . Gentle airway instrumentation was performed to pass 7.5 no. Portex cuffed endotracheal tube. Airway leak was obliterated by minimal inflation of the cuff. Nasogastric aspiration tube and endotracheal tube were fixed with single strip of elastic adhesive bandage. Surgery lasted for eight hours. All vital parameters were well maintained intraoperatively. There was 5 liters of blood loss which was adequately and immediately replaced. Intraoral low suction was resorted to only when required. Postoperatively she was put on prophylactic ventilator therapy and shifted out of the intensive care unit in 48 hours. Steroid cover was given perioperatively.
The skin over the face was not affected by the anaesthetic maneuvers intraoperatively and in the immediate postoperative period. However mild ecchymosis was seen around the cannulae site which was treated with calamine lotion. The ecchymotic lesions disappeared in 7 days.
The final diagnosis of the tumor was Castleman's disase of the hyaline vascular type.
| Discussion|| |
The major problems involved in anaesthetizingpemphigus patients relate to adrenocortical suppression induced by prolonged steroid therapy and to the possibility of causing worsening of pemphigus ,. Increased activity of the pemphigus lesions may be triggered by stress, trauma and by irritation of the skin and the mucus membranes by the use of face masks, airway and tracheal tubes.
Evidence of the diseases associated with paraneoplastic pemphigus such as rheumatoid arthritis and myasthenia gravis must be sought and recognized in the preoperative assessment. Each disease requires additional considerations for anaesthetic management. ,,,
No particular anaesthetic technique has been recommended in patients with pemphigus. But whenever possible regional anaesthesia is preferable to general anaesthesia  .Local infiltration is contraindicated because of the potential danger of sloughing. Airway management may be difficult because of cutaneous, oropharyngeal and laryngeal involvement. Whenever general anaesthesia requiring intubation is unavoidable , , all measures to protect skin surfaces must be taken. Airway instrumentation should be cautious and gentle. Perioral scarring may restrict opening of the mouth. The use of indwelling temperature probes and stethoscopes should be avoided. Intravenous and arterial lines should be secured in place by suture and gauze bandaging , . Eyes should be protected with ophthalmic ointment but no tape should be applied. Skin denudation and bullae formation can result in significant fluid and protein loss  . Local skin care with wet compresses helps to prevent skin trauma. Severe oropharyngeal lesions may interfere with adequate nutrition. Lesions of pharynx and larynx may produce hoarseness . Electrolyte imbalance and hypoalbuminaemia is present in 5 -6 % of cases of pemphigus. Despite the rarity of reports of post extubation airway problems from bullae or haemorrhage , these complications are grave and all patients must be carefully observed in the postoperative period for any airway problems.
Paraneoplastic pemphigus is associated with an underlying neoplasm , either malignant or benign . Thus the exclusion of occult neoplasm is important in suspected cases of paraneoplastic pemphigus especially by computerized tomography of the chest, abdomen and pelvis , . Patients with paraneoplastic pemphigus and benign tumors ( such as thymoma or Castleman's ) should have the tumor surgically excised. The majority of the patients improve substantially or completely. Complete resolution of skin and mucus membrane lesions usually occurs 6 - 18 months after excision of benign neoplasm.
In our case we had to resort to general anaesthesia in view of the site of surgery. Other associated conditions like rheumatoid arthrits , myaesthenia gravis and pernicious anaemia were ruled out. Special precautions were taken in securing intravenous lines and endotracheal tube. Endotracheal intubation was performed gently and preparations were made to handle any untoward incident should it occur.
We feel endotracheal intubation should be undertaken with utmost care to prevent any exacerbation of pemphigus state when prolonged surgery is unavoidable  .
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