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CLINICAL

A surprising presentation of blistering ulcers

Karen Broadbent

Case and target lesions over the abdomen, can include drug therapy and recent viral A man, 28 years of age, presented to an genital region and upper limbs (Figure 2). , and it is commonly associated with malaise, fever and joint pains. emergency department with a seven-day Question 1 history of lethargy, which he attributed vulgaris is a rare autoimmune to the recent stress of working on a What are the possible diagnoses? disease that is characterised by on university thesis. The day before, he the and mucous membranes, usually Question 2 developed what he described as mouth starting in the mouth. ulcers, which progressively became more What is the pathophysiology of the always has a positive Nikolsky sign, which painful, causing some dysphagia. diagnoses? is the dislodgement of intact superficial He had previously been prescribed by a shearing force, resulting in modafinil for narcolepsy, although he took Question 3 exfoliation of the outermost layer forming a this intermittently while studying. His last What is the most likely cause of this within minutes.1–3 dose of modafinil was four days before he condition? Herpetic gingivostomatitis is an developed the mouth ulcers. He had no inflammation of the and other significant past history and was on Answer 1 gingiva, predominantly caused by no other medications. On examination, The possible diagnoses for this patient are: the , that mainly there were white sloughy blisters in the • autoimmune disorder (eg affects children. is another labial mucosa and raised ulcerative areas vulgaris) oral ulcerative condition caused by the in the hard . There were also four • herpetic gingivostomatitis .4,5 well-circumscribed, firm blisters in the • Stevens–Johnson syndrome (SJS). Stevens–Johnson Syndrome (SJS) buccal mucosa. Systemically, he was well is an immune complex-mediated and there were no adverse findings on Answer 2 hypersensitivity disorder that may be thorough examination. Autoimmune disorders (eg pemphigus caused by drugs, viral infections or Blood profiles were taken for vulgaris) occur when the body’s immune malignancies, or it can be idiopathic. SJS haematology, biochemistry and system attacks and destroys healthy body is characterised by confluent epidermal inflammatory markers, which returned tissue. Causes of autoimmune disorder necrosis with minimal associated within acceptable parameters. A blister was aspirated for virology, and for microscopy, culture and sensitivity (MC&S). He was discharged at the time, instructed to stop taking the modafinil and requested to return to the clinic the next morning. Due to university stressors, he did not return until two days later when he developed odynophagia, sloughing of the labial mucosa, crusty haemorrhagic lesions Figure 1. Sloughing of the labial mucosa Figure 2. Target lesion on finger over the upper and lower (Figure 1)

118 REPRINTED FROM AFP VOL.45, NO.3, MARCH 2016 © The Royal Australian College of General Practitioners 2016 BLISTERING MOUTH ULCERS CLINICAL

inflammation caused by cell death, forcing Answer 4 Competing interests: None. the epidermis to separate from the Treatment is usually symptomatic, with Provenance and peer review: Not commissioned, externally peer reviewed. . SJS is considered a milder form of careful attention paid to airway management toxic epidermal necrosis (TEN). and haemodynamic stability. Therapy for a References SJS and TEN are differentiated by using patient with SJS would include, but is not 1. Scully C, Paes De Almeida O, Porter SR, Gilkes JJH. Bastuj’s classification, which is based on limited to: Pemphigus vulgaris: The manifestations and long- term management of 55 patients with oral lesions. the type of lesions, extent of blisters and • withdrawal of the offending drug – certain Br J Dermatol 1999;140:84–89. erosions on the total body surface. Blisters medications have been linked to SJS 2. British Skin Foundation. Pemphigus vulgaris. London: British Skin Foundation, 2015. Available at and erosions cover 3–10% of the body in and should be avoided (eg modafinil, www.britishskinfoundation.org.uk/SkinInformation/ SJS, 10–30% in overlapping SJS/TEN and nonsteroidal anti-inflammatory drugs AtoZofSkindisease/Pemphigusvulgaris.aspx more than 30% in TEN.6–8 and sulphur-based medication).6–8,12 The [Accessed 19 November 2014]. 3. Guillen S, Khachemoune A. Pemphigus vulgaris: SJS generally starts with an insidious patient should be strongly advised to stop A short review for the practitioner. Dermatol Nurs onset of non-specific, upper respiratory using the offending drug and consider 2007;19:269–72. tract , then a 1–14-day prodrome 4. Kolokotroni A, Doumas S. Herpes simplex wearing a MedicAlert bracelet virus infection, with particular reference to of fever, , diarrhoea, headache • admission to hospital considering the the progression and complications of primary and fatigue, with mucosal membrane vulnerability of the patient’s airway and herpetic gingivostomatitis. Clin Microbiol Infect 2006;12:202–11. lesions developing, followed by possibility of compromise 5. Lab Tests Online. Herpes testing. Washington, DC: lesions over the torso and genital area. • analgesia – a topical local anaesthetic American Association for Clinical Chemistry, 2013. Conjunctivitis occurs in approximately Available at http://labtestsonline.org/understanding/ such as mouth irrigation with viscous analytes/herpes/tab/sample [Accessed 30 July 30% of children and 3–10% of adults lignocaine gel can give relief to allow 2015]. with SJS.6 oral analgesia to be taken; however, care 6. Tyagi S, Kumar S, Kumar A, Singla M, Singh A. Stevens–Johnson syndrome – A life threatening The resembles a target, which is must be taken to avoid local anaesthetic skin disorder: A review. J Chem Pharm Res considered pathognomonic. Mortality is toxicity11,12 2010;2:618–26. determined primarily by the extent of skin 7. Knepel S. multiform, Stevens–Johnson • mouth care – mouth lesions have been syndrome and toxic epidermal necrolysis. Atlanta, sloughing. When total body surface area known to erupt for as long as 14–21 days GA: AHC Media, 2015. Available at http://www. sloughing is less than 10%, the mortality and can extend into the oesophagus, with ahcmedia.com/articles/16029-erythema-multiforme- rate is approximately 1–5%. However, stevens-johnson-syndrome-and-toxic-epidermal- mucosal shedding in the tracheobronchial necrolysis [Accessed 7 December 2015]. when more than 30% is present, the tree, leading to respiratory failure6–8 8. Knoop KJ, Stack LB, Storrow AB, Thurman RJ. The 6–9 atlas of emergency medicine. 3rd edn. New York: mortality rate is 25–35%. It has a • urgent dermatology review 7, 8 McGraw-Hill, 2010. positive Nikolsky sign. • chest X-ray to exclude pneumonitis4 9. Cheriyan S, Patterson R, Greenberger PA, Grammer • fluid intake – fluid resuscitation with LC, Latall J. The outcome of Stevens– Johnson Answer 3 syndrome treated with corticosteroids. Allergy Proc correction of electrolyte imbalance 1995;16:151–54. The most likely cause is modafinil, • tetanus prophylaxis 10. Food and Drug Administration. Protecting which has been documented as a • treatment as per the burns protocols if and promoting your health. Washington, DC: FDA, 2013. Available at www.fda. catalyst in SJS.6,9 It is a psychostimulant, there are any external skin eruptions gov/Safety/MedWatch/SafetyInformation/ wakefulness medication that is available • use of corticosteroids – this is SafetyAlertsforHumanMedicalProducts/ucm152701. on the Pharmaceutical Benefits Scheme htm [Accessed 30 July 2015]. controversial and some authors report 11. Department of Health. Pharmaceutical Benefits (PBS), although authority is required by that use of corticosteroids can increase Scheme. Canberra: DoH, 2015. Available at www. pbs.gov.au/pbs/home [Accessed 10 February 2015]. a qualified sleep medicine practitioner or the length of hospital stay, increase neurologist. The direct action of the drug is 12. Rang HP, Dale MM, Ritter JM, Flower RJ. Rang the rates of infection and lead to more and Dale’s pharmacology. 6th edn. China: Churchill unclear, but it is thought that it alters the complications3,6,7,9,13–16 Livingstone, 2008. neurotransmitters in the brain.10,11 13. Rasmussen JE. in children. • ophthalmology review – ocular sequelae Response to treatment with systemic steroids. Br J Case continued can include corneal ulceration and anterior Dermatol 1976;95:181–86. uveitis.6 Blindness, secondary to severe 14. Kardaun S, Jonkman M. Dexamethasone A tissue was taken from a target pulse therapy for Stevens–Johnson syndrome/ keratitis or panophthalmitis, may develop toxic epidermal necrolysis. Acta Derm Venereol lesion and sent for histology. It confirmed in 3–10% of affected patients. The use of 2007;87:144–48. 15. Kakourou T, Klontza D, Soteropoulou F, Kattamis C. a diagnosis of SJS. The method used lubricating eye ointments is advocated. direct to exclude Corticosteroid treatment of erythema multiforme major (Stevens–Johnson syndrome) in children. Eur pemphigus. Author J Pediatr 1997;156:90–93. Karen Broadbent DipAsthma (NARTC), DipDiabetes, 16. Michaels B. The role of systemic corticosteroid Question 4 PGradDipClinNsg, MN,RN, Emergency Nurse therapy in erythema multiforme major and Stevens– Practitioner, Fiona Stanley Hospital, Perth, WA. Johnson syndrome – A review of past and current What is the management for this patient? [email protected] opinions. J Clin Aesthet Dermatol 2009;2:51–55.

© The Royal Australian College of General Practitioners 2016 REPRINTED FROM AFP VOL.45, NO.3, MARCH 2016 119