The Journal of Emergency Medicine, Vol. -, No. -, pp. 1–2, 2015 Copyright Ó 2015 Elsevier Inc. Printed in the USA. All rights reserved 0736-4679/$ - see front matter
Visual Diagnosis in Emergency Medicine
ERYTHEMA MULTIFORME Eric T. Stoopler, DMD, FDS RCSED, FDS RCSENG, Alicia M. Houston, DDS, Milda Chmieliauskaite, DMD, MPH, and Thomas P. Sollecito, DMD, FDS RCSED Department of Oral Medicine, University of Pennsylvania School of Dental Medicine, Philadelphia, Pennsylvania Corresponding Address: Eric T. Stoopler, DMD, FDS RCSED, FDS RCSENG, University of Pennsylvania School of Dental Medicine, 240 S 40th St, Philadelphia, PA 19104
2 weeks (Figure 2). At the present time, the patient is continuing with chronic HSV prophylaxis.
CASE REPORT A 25-year-old woman presented in distress to an oral medicine clinic for evaluation of recurrent oral lesions of 3 years duration. Occurring at least 3 times annually, she reported an acute onset of diffuse oral ulcerations accompanied by hemorrhagic crusting and swelling of the lips that resolved within 4 weeks. She denied extraoral lesions, a history of clinical herpes simplex virus (HSV) infection, and was unaware of any first-degree relatives with a history of oral ulcers. The patient was evaluated in different emergency departments on 5 separate occasions and was diagnosed with aphthous stomatitis or HSV infection. The patient was routinely prescribed topical anesthetics and valacyclovir 1000-2000 mg daily without benefit. The clinical examination revealed diffuse, erythematous, ulcerative lesions on the tongue and oral mucosa without evidence of cutaneous lesions. Hemorrhagic crusting and edema was evident on both the upper and lower lips (Figure 1). The diagnosis was consistent with erythema multiforme (EM) minor, and the patient was treated with methylprednisolone 24 mg (tapering dose), viscous lidocaine 2%, and valacyclovir 500 mg daily for HSV prophylaxis with resolution of the lesions within
DISCUSSION EM is an immune-mediated hypersensitivity reaction most commonly seen in patients between 20 and 30 years of age, and it has a possible female predilection (1–4). Infectious agents are the most common trigger for EM, with HSV-1 implicated in > 70% of cases (1–5). Other common infectious agents include HSV-2 and Mycoplasma pneumoniae (1–4). Several medications are considered highly suspicious for triggering episodes of EM, including sulfonamides, nonsteroidal antiinflammatory drugs (NSAIDs), and penicillins (1–4). EM is primarily diagnosed on a clinical basis and is classified as minor or major based upon specific clinical criteria. Both types typically feature cutaneous lesions that are often described as ‘‘targetoid’’ in appearance, although the patient denied the presence of cutaneous lesions in this case. Diffuse, nonspecific, erythematous oral ulcers with edematous, hemorrhagic-encrusted lips are common to both variants (1–4). Mucosal involvement is uncommon in the minor type; however, if mucosal involvement is present, it typically affects the mouth. EM minor frequently has an acute onset without prodromal symptoms. EM major consists of $2 involved mucosal sites, with the mouth usually affected. In contrast to the
Reprints are not available from the authors.
RECEIVED: 26 May 2015; ACCEPTED: 4 June 2015 1
E. T. Stoopler et al.
Figure 1. Hemorrhagic, edematous lips with tongue ulcers consistent with erythema multiforme minor.
minor variant, EM major is generally more severe, widespread, and associated with prodromal symptoms before the onset of cutaneous and mucosal lesions (1–4). Management strategies for EM are based on the etiology, severity, and frequency of episodes. If an infectious trigger is identified, appropriate medical therapy is indicated. Patients are often empirically prescribed chronic antiviral prophylaxis for HSV-induced EM, as was done in this case. Specific medications should be avoided if they are suspected triggers of EM. Patients with mild oral disease may be treated with topical corticosteroids, such as fluocinonide gel 0.05% twice daily until lesions resolve (1–4). Severe EM is managed with short-term systemic corticosteroids and immunosuppressants for recurrent disease, such as azathioprine and mycophenolate mofetil (1–4).
Figure 2. Resolution of erythema multiforme minor after treatment with systemic corticosteroids, viscous lidocaine, and prophylactic valacyclovir.
It is important for health care providers to distinguish EM from other mucocutaneous disorders to appropriately diagnose and effectively manage patients with this condition. REFERENCES 1. Al-Johani KA, Fedele S, Porter SR. Erythema multiforme and related disorders. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007; 103:642–54. 2. Schifter M, Yeoh SC, Coleman H, Georgiou A. Oral mucosal diseases: the inflammatory dermatoses. Aust Dent J 2010;55(suppl 1):23–38. 3. Williams PM, Conklin RJ. Erythema multiforme: a review and contrast from Stevens-Johnson syndrome/toxic epidermal necrolysis. Dent Clin North Am 2005;49:67–76:viii. 4. Samim F, Auluck A, Zed C, Williams PM. Erythema multiforme: a review of epidemiology, pathogenesis, clinical features, and treatment. Dent Clin North Am 2013;57:583–96. 5. Aurelian L, Kokuba H, Burnett JW. Understanding the pathogenesis of HSV-associated erythema multiforme. Dermatology 1998;197:219–22.