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 Table of Contents  
Year : 2022  |  Volume : 1  |  Issue : 2  |  Page : 100-102

Anesthetic management of a patient with erythema multiforme for emergency appendectomy

Department of Anaesthesia and Intensive Care, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India

Date of Submission02-Nov-2022
Date of Decision13-Nov-2022
Date of Acceptance14-Nov-2022
Date of Web Publication02-Dec-2022

Correspondence Address:
Dr. Mousumi Saha
Department of Anaesthesia and Intensive Care, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi - 110 002
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jica.jica_31_22

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Erythema multiforme (EM) is an acute, immune-mediated, mucocutaneous disease, caused by several factors such as infections, drugs, malignancy, autoimmune disease, radiation, and immunization. The anesthetic concerns in these patients include possibility of difficult airway, intraoperative steroid supplementation, avoidance of precipitating drugs, intraoperative arrhythmias, perioperative skin, and eye care. A 13-year-old boy, a known case of EM with a history of recurrent ocular herpes and impaired vision along with fibrosis at the bilateral angle of the mouth underwent emergency open appendectomy under spinal anesthesia. Spinal anesthesia with injection hyperbaric bupivacaine (0.5%) is a safe and suitable alternative in a patient with EM with recurrent herpes simplex virus.

Keywords: Erythema multiforme, herpes simplex virus, Steven–Johnson syndrome

How to cite this article:
Saha M, Mehra A, Saxena KN, Wadhwa B. Anesthetic management of a patient with erythema multiforme for emergency appendectomy. J Ind Coll Anesth 2022;1:100-2

How to cite this URL:
Saha M, Mehra A, Saxena KN, Wadhwa B. Anesthetic management of a patient with erythema multiforme for emergency appendectomy. J Ind Coll Anesth [serial online] 2022 [cited 2023 Oct 1];1:100-2. Available from: https://www.jicajournal.in//text.asp?2022/1/2/100/362617

  Introduction Top

Erythema multiforme (EM) is an acute, immune-mediated, mucocutaneous hypersensitivity reaction of variable severity characterized by acrally distributed skin lesions, sometimes accompanied by oral, genital, or ocular mucosal erosions or a combination of these.[1],[2],[3] Patients with EM presenting for emergency surgery may pose great challenges to anesthesiologists such as difficult airway, dyselectrolytemia, and malnutrition cause by recurrent extensive skin and mucosal lesions, risk of life-threatening hypersensitivity reactions with precipitating drugs, coexisting myocarditis.[4] We report the successful anesthetic management of a 13-year-old male patient, known case of EM major for the last 4 years with a history of recurrent ocular herpes, impaired vision and limited mouth opening due to fibrosis at the bilateral angle of the mouth who presented for an emergency open appendectomy.

  Case Report Top

A 13-year-old boy of weight 45 kg and height of 150 cm, a known case of EM for the last 4 years presented for emergency open appendectomy. Preanesthetic check-up revealed a history of recurrent ocular herpes with impaired vision along with multiple skin, oral mucosal ulcerative lesions and fibrosis at the bilateral angle of the mouth leading to reduced mouth opening. The patient was on treatment with tablet acyclovir 200 mg five times a day along with steroid supplementation. While there was no active skin lesion, there were oral mucosal ulcers. On airway examination, mouth opening was restricted to 1.5 cm, Modified Mallampati class IV with fibrous web formation at bilateral angle of mouth because of recurrent angular ulceration. All the preoperative blood investigations, 12 lead electrocardiogram, chest X-ray were normal. Echocardiography was not possible due to time constraints in view of the emergency nature of surgery. We decided for spinal anesthesia after the examination of the local site for the absence of skin lesions. Epidural anesthesia was our rescue plan in case of failed spinal anesthesia. High risk written informed consent was taken in view of anticipated difficult airway and risk of life-threatening perioperative drug hypersensitivity reaction. The patient was taken inside the operation theater after keeping the difficult airway cart and drugs to manage any hemodynamic instability (inotropes, vasopressor, antiarrhythmic, etc.) ready. After attaching all ASA standard monitors, baseline vitals were noted. Following that premedication was done with injection hydrocortisone 100 mg (for steroid supplementation), injection dexamethasone 4 mg (as part of multimodal analgesia and antiemetic prophylaxis), injection ranitidine 50 mg and injection midazolam 0.5 mg intravenously (i.v.). After preloading with 500 ml RL, subarachnoid block was administered with 0.5% injection bupivacaine (hyperbaric) 1.8 ml and injection fentanyl 10 μg at L3–L4 level using 27G Whitacre spinal needle and a sensory level of T6 was achieved. The patient was hemdynamically stable throughout the surgery which lasted for 80 min. Injection paracetamol 15 mg/kg body weight i.v. every 6 hourly was advised for postoperative analgesia. Injection diclofenac was avoided in view of potential to cause drug hypersensitivity reaction in such patients. Both intraoperative and postoperative periods were uneventful.

  Discussion Top

Von Hebra first described the disease in 1866 as acrally distributed typical target lesions or raised edematous skin papules with or without mucosal involvement.[5] The incidence of EM is very less (0.01%–0.1%) in the general population, majority being seen in individuals between 20 and 40 years of age with male preponderance. The disease is recurrent in approximately 30% of cases.[6] The leading causes are drugs (nonsteroidal anti-inflammatory drugs, sulfonamides, penicillins, cephalosporins, erythromycin, tuberculostatics, salicylates, pyrazolones, carbamazepine, phenobarbital, and phenytoin), infections (viral, bacterial, fungal, parasitic, and mycoplasma infections), endocrine factors, neoplasms. Ninety percent of cases are caused by infections, most common being herpes simplex virus (HSV) (HSV1 > HSV2) and drug-associated EM is reported in <10% of cases. Recurrent EM seems to be associated with the HLA-B35, HLA-B62, 15 and HLA-DR5320 alleles and is thought to result from cell-mediated immune reaction against HSV antigen-positive cells.[3]

Based on the severity and the number of mucosal sites involved, it can be of two types EM minor and major. EM minor shows ulcerations involving a single mucosal site with typical skin target lesions. EM major is considered to be a more aggressive form characterized by involvement of multiple mucosa accompanied by typical target skin lesions. The third category of EM, also described by many investigators as oral EM has the lesions confined to the oral mucosa and lips with no skin involvement. Stevens–Johnson syndromes (SJS) is an extreme form of EM in which there is extensive acute inflammation of the skin and mucous membranes characterized by cutaneous desquamation with a higher mortality rate than EM (3%–18%). Patients need urgent definitive airway management and ventilatory and circulatory support.[4] Our patient had EM major caused by HSV1 for the last 4 years and was suffering from recurrent ocular herpes with impaired vision along with multiple skin, oral mucosal ulcerative lesions without any history of fulminant SJS in past.

There is a paucity of the literature about the anesthetic management of the patients with EM with no past history of SJS and any drug hypersensitivity reactions posted for emergency surgery. While assessing and managing a patient with EM, the following things should be considered: Evaluation of duration, severity and cause of the disease and documentation of drug hypersensitivity reaction in past should be done. Pre-operative optimization for hypovolemia and dyselectrolytemia in fulminant cases with extensive acute lesions is needed. Pre-habilitation in severe chronic cases that may present with cachexia and malnutrition should be considered whenever possible. Careful cardiac evaluation is must to rule out any arrhythmia as a sign of underlying myocarditis seen in these patients with EM. Also consider preoperative echocardiography to define ventricular function and quantify pericardial effusion in case of suspected myocarditis whenever possible. Planning of anaesthesia should be done after proper assessment of above and after examination of airway and lesions of skin and mucous membrane. Care should be taken while handling patient and applying monitoring devices in view of incomplete skin barrier to avoid contamination and further development of infection and sepsis. Proper care of eyes is to be taken to protect them in case of ocular involvement. Consider perioperative corticosteroid supplementation since these patients may be on chronic corticosteroid therapy with pituitary adrenal suppression. While intubating the patient, gentle laryngoscopy and minimum airway manipulation are desirable if active airway lesions are suspected. Anaesthesiologist must be vigilant for any intraoperative drug hypersensitivity reactions and should be prepared to tackle such an event.[6] Drugs commonly used in anaesthesia such as barbiturate, lignocaine glycopyrrolate, midazolam, ondansetron, fentanyl, and neostigmine have rarely been reported to precipitate SJS/EM and so should be avoided.[7],[8]

Challenges while administering general anesthesia are anticipated difficult airway due to recurrent oral mucosal ulcers forming fibrous band, hypersensitivity reactions to commonly used anaesthetic agents as mentioned above, use of nitrous oxide may cause rupture of underlying bulla leading to occult barotrauma, contamination of anesthesia machine. For these reasons, regional anesthesia is preferred over general anesthesia wherever possible as long as skin at the site of the block is normal.[4],[9],[10]

In our case, patient had EM major caused by HSV1 for the last 4 years with anticipated difficult airway (fibrous web at the bilateral angle of the mouth restricting mouth opening to 1.5 cm). He had no past history of fulminant SJS and any anesthesia exposure and any drug hypersensitivity reactions. Spinal anesthesia was administered successfully using injection hyperbaric bupivacaine (0.5%) while remained continuous vigilant for perioperative drug hypersensitivity reaction. All the precautions were taken to avoid patient's exposure to offending drugs. We also kept difficult airway cart (smaller sized endotracheal tube, stylet, bougie, video laryngoscope, fiber optic bronchoscope, and supraglottic airway devices) and all the drugs required to manage any hemodynamic compromise, ready for the emergency.

With that we conclude that spinal anesthesia with hyperbaric bupivacaine (0.5%) can be administered safely in a known case of EM major with recurrent HSV infection undergoing emergency open appendectomy under close perioperative monitoring for any drug hypersensitivity reaction with all pharmacological agents and difficult airway cart ready in hand to tackle the situation promptly.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Assier H, Bastuji-Garin S, Revuz J, Roujeau JC. Erythema multiforme with mucous membrane involvement and Stevens-Johnson syndrome are clinically different disorders with distinct causes. Arch Dermatol 1995;131:539-43.  Back to cited text no. 1
French LE, Prins C. Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Dermatology. 2nd ed. Mosby: Elsevier, St Louis; 2008. p. 287-300.  Back to cited text no. 2
Sokumbi O, Wetter DA. Clinical features, diagnosis, and treatment of erythema multiforme: A review for the practicing dermatologist. Int J Dermatol 2012;51:889-902.  Back to cited text no. 3
Smith GB, Shribman AJ. Anaesthesia and severe skin disease. Anaesthesia 1984;39:443-55.  Back to cited text no. 4
Joseph TI, Vargheese G, George D, Sathyan P. Drug induced oral erythema multiforme: A rare and less recognized variant of erythema multiforme. J Oral Maxillofac Pathol 2012;16:145-8.  Back to cited text no. 5
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BaniHani A, Nazzal H, Webb L, Toumba KJ, Fabbroni G. An unusual presentation of erythema multiforme in a paediatric patient. Eur Arch Paediatr Dent 2015;16:297-302.  Back to cited text no. 6
Chowdhury D, Khatavkar S, Samuel M. A rare case of Stevens-Jonhson syndrome with attention deficit hyperkinetic disorder and seizure disorder for circumcision: An anesthetic challenge. Med J DY Patil Univ 2017;10:64-6.  Back to cited text no. 7
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Rodríguez-Carreón AA, Vega-Memije E, Moreno-Coutiño G, López-García L, Domínguez-Cherit J, Arenas R. Simultaneous erythema nodosum and erythema multiforme after local lidocaine injection. Ann Pharmacother 2008;42:127-30.  Back to cited text no. 8
Gupta LK, Martin AM, Agarwal N, D'Souza P, Das S, Kumar R, et al. Guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis: An Indian perspective. Indian J Dermatol Venereol Leprol 2016;82:603-25.  Back to cited text no. 9
[PUBMED]  [Full text]  
Parashar VK, Mitharwal SM, Chaudhary A. Anesthetic considerations in Stevens-Johnson syndrome with epilepsy for bilateral amniotic membrane grafting in eye. Indian J Anaesth 2018;62:569-70.  Back to cited text no. 10
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