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Year : 2007  |  Volume : 51  |  Issue : 6  |  Page : 525-527 Table of Contents     

Anaesthetic management for paraneoplastic pemphigus

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 Acceptance20-Sep-2007
Date of Web Publication20-Mar-2010

Correspondence Address:
S T Dave
4/C, BINDIYA, Reclamation area, Bandra (West), Mumbai - 400050
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Source of Support: None, Conflict of Interest: None

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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 2021 Jun 25];51:525-7. Available from: https://www.ijaweb.org/text.asp?2007/51/6/525/61192

   Introduction Top

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 [1] . 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 [2] .

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 Top

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 Top

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 [1],[2]. 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. [3],[4],[5],[6]

No particular anaesthetic technique has been rec­ommended in patients with pemphigus. But whenever possible regional anaesthesia is preferable to general anaesthesia [7] .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 [8],[9] , 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 [10],[11] . 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 [12] . 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 [5],[6] . 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 [13] .

   References Top

1.Rosenberg FR, Sander S, Nelson CT. Pemphigus, A 20 year review of 107 patients treated with corticosteroid. Archive of Dermatology 1976 ; 112 : 962-70  Back to cited text no. 1      
2.Lever WF, Schaumburg-Lever G. Immunosuppressants and prednisone in pemphigus vulgaris. Archive of Dermatology 1977 ; 113 : 1236  Back to cited text no. 2      
3.Chryssomallis F, Dimitriades A, Chaidemenos GC, et al. Ste­roid pulse therapy in pemphigus vulgaris long term follow up. Internal Journal of Dermatology 1995 ; 34 : 438  Back to cited text no. 3      
4.Krain LS, Bierman SM. Pemphigus vulgaris and internal malig­nancy. Cancer 1974; 33 : 1091  Back to cited text no. 4      
5.Naysmith A, Hancock BW. Hodgkins disease and pemphigus. British Journal of Dermatology 1976 ; 94 : 696  Back to cited text no. 5      
6.Krain LS.The association of pemphigus thymoma or malig­nancy. Critical review, British Journal of Dermatology 1974 ; 90 : 397  Back to cited text no. 6      
7.Abouleish EI, Elias MA, Lopez M, et al. Spinal anaesthesia for caesarean section in a case of pemphigus foliaceus.Anesthesia & Analgesia 1997 ; 84 : 449-50  Back to cited text no. 7      
8.Jeyaram C, Torda TA. Anaesthetic management of cholecys­tectomy in a patient with buccal pemphigus. Anesthesiology 1975 ; 40 : 600-01  Back to cited text no. 8      
9.Vatashsky E, Aronson HB. Pemphigus vulgaris, anaesthetic management in the traumatised patient. Anaesthesia 1982 ; 37 : 1195-97  Back to cited text no. 9      
10.Arif S, Kaye AD. Pre-anesthetic management in a patient with pemphigus. Anesthesiology News 1999 ; 25 : 8-12  Back to cited text no. 10      
11.Prasad KK, Chen L. Anesthetic management of a patient with bullous pemphigoid. Anesthesia Analgesia 1989 ; 69 : 537-40  Back to cited text no. 11      
12.Brain L Partridge. Skin and bone disorders in BL Partridge, JL Benumoff authored Benumoff, Anesthesia and Uncommon diseases 1998 ; 4th edition, chapter12 ,426-428.  Back to cited text no. 12      
13.Drenger B, Zidenbaum M, Reifen E, et al. Severe upper airway obstruction and difficult intubation in cicatricial pemphigoid. Anaesthesia 1986 ; 41 : 1029-31.  Back to cited text no. 13  [PUBMED]    


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