Erythema Multiforme: A Case Report

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Case Report

Erythema Multiforme: A Case Report Arutla Rashmitha, Srikanth G Gotoor, Srikar Muppirala, Devaraju R Raju Department of Oral Medicine and Radiology, Sri Venkata Sai Institute of Dental Sciences, Mahabubnagar, Telangana, India

Abstract Erythema multiforme (EM) is a life threatening mucocutaneous disorder where early diagnosis and management is of utmost important. Among various etiological agents, viral etiology is the most common and is characterized by ulcerations, erosions, and bleeding within the mucosa associated with encrustations and tissue tags. Dermal counterparts present with target iris lesion. Here, we report a case of viral‑induced EM major where there are classical oral and dermal lesions in a 28‑year‑old male patient. Keywords: Antiviral, erythema multiforme, target iris lesions

Introduction Erythema multiforme (EM) is an intense, self‑limiting, recurrent mucocutaneous disorder that manifests with a reaction pattern which appears as a consequence of allergic host response to antigenic challenge.[1] EM is triggered primarily by the antigens, which are induced by exposure to microbes or by certain medication.[2] Viral etiology is the most common.[3]

Case Report A 27‑year‑old male presented with a painful mouth owing to ulcers associated with bleeding since 2 weeks. History revealed fever and body pains 2 weeks ago, following which he noticed vesicles on the bilateral buccal mucosa and labial mucosa. The vesicles subsequently ruptured leaving ulcerated areas associated with severe pain and bleeding on mastication, making it difficult to consume both solid and liquid foods. Five days after the inception of oral lesions, he developed cutaneous lesions on the upper and lower extremities, which were preceded by itching. He consulted a private practitioner for the same reason and was referred to higher centers. General physical examination unveiled multiple concentric target or iris lesions on upper and lower extremities. Encrustations that bleed readily on provocation were noticed on the lips and upper and lower labial mucosa which were jagged and tender, sparing gingiva. Compromised oral hygiene, diffuse multiple ulcers, and erosions were present on the upper and lower labial mucosa, bilateral buccal mucosa extending to Access this article online Quick Response Code:

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DOI: 10.4103/jiaomr.JIAOMR_168_16

the retromolar area [Figure 1]. The floor of ulcers was covered by pseudomembrane, surrounded by erythematous halo with irregular margins and a nonindurated base with bleeding on provocation. Considering the history, prodromal symptoms, hemorrhagic crustations involving the upper and lower lips in addition to widespread involvement of oral mucosa and symmetric distribution of the target/iris/bull eye shaped dermal lesions (pathognomic) on extremities [Figure 2], the condition was diagnosed as EM major. Clinical differential diagnosis included herpetic gingivostomatitis (affects children and teenagers) and mucous membrane pemphigoid and pemphigus vulgaris (primarily affects the gingiva and are chronic). The patient was commenced on systemic corticosteroid prednisolone 20 mg b.i.d., tab acyclovir 400 mg t.i.d., oint. triamcenalone acetonide 0.1% t.i.d., along with benzydamine hydrochloride mouthwash for a week. As the lesions were regressing clinically with gradually decreasing discomfort after 15 days [Figures 3 and 4], systemic steroids were tapered by 10 mg for every week till a maintenance dose of 5 mg. The patient was subjected to quadrant scaling, and oral hygiene Address for correspondence: Dr. Srikar Muppirala, Department of Oral Medicine and Radiology, Sri Venkata Sai Institute of Dental Sciences, Appanapally, Mahabubnagar, Telangana, India. E‑mail: [email protected] This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. For reprints contact: [email protected]

How to cite this article: Rashmitha A, Gotoor SG, Muppirala S, Raju DR. Erythema multiforme: A case report. J Indian Acad Oral Med Radiol 2017;29:153-5. Received: 27-12-2016   Accepted: 24-10-2017   Published: 09‑11‑2017

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Figure 1: Series of pictures depicting multiple encrustations, and ulcerations, with clinically evident bleeding Figure 2: Pictures showing target or iris lesions on extremities

Figure 3: Series of intraoral photographs showing partially regressed lesions after 1 week under medication

instructions were given. Total resolution of the oral and dermal lesions was attained after 1 month [Figures 5 and 6]. The patient is currently under review with no recurrence at 1 year follow‑up.

Discussion

Figure 4: Regressing target lesions on extremities

such as macrophages and CD34+ Langerhans cell progenitors phagocytose the virus in peripheral blood, which have skin homing receptor cutaneous lymphocyte antigen. The engulfed HSV DNA is transported to the epidermis, where the fragmented viral DNA is transferred to the keratinocytes. Upregulation of E‑cadherin expression increases the binding of HSV‑containing Langerhans cells to the endothelial cells. In addition, adhesion molecule upregulation on endothelial cells accounts for the inflammatory response.[3] On the contrary, drug‑associated EM seems to involve CD8+ T‑cell attack and expression of TNF‑α in the lesion.[7]

It dates back to 1866 where Von Hebra first mentioned the acral distribution of target iris lesions in EM with or without mucosal involvement.[4] Classification of EM is according to the degree of mucosal involvement and existence of dermatologic lesions. The most frequent form is EM minor that affects only one mucosa and may be associated with acrally distributed proportioned target or iris lesions. In EM major symmetrical dermal target lesions and at least two mucosal sites are affected.[5] Medical literature has coupled numerous factors to the development of EM. These include infections, use of certain medications, malignancy, autoimmunity, radiation, immunization, and menstruation. Of these, infection represents approximately 90% cases, and the most common agent is herpes simplex virus (HSV),[3] which is drawn in up to 70–80% of the cases.[2,6]

EM usually affects individuals at 20 to 40  years. The oral lesions are accompanied by swiftly rupturing vesicles and bullae, leading to diffuse sloughing and ulceration of the entire surface of the mucous membrane. Acute repeated ulceration can add to systemic upset and possibly a compromised life.[6,8,9] The chair side features are consistent with that of the earlier literature documentations. HSV as an etiologic agent in the etiological spectrum of EM can be further reinforced with the present case.

Mechanisms unfolding the pathogenesis of EM have been based on analytical studies of HSV‑associated EM. It is thought to be the consequence of cell‑mediated immune reaction against viral antigen positive cells containing the HSV DNA polymerase gene (pol). The mononuclear cells

There is no precise diagnostic investigation for EM. The vital clues to diagnosis continue to be the clinical history and clinical findings. Significant components of the history with: (1) an acute, self‑limiting or periodic course, (2) signs and symptoms of allied infections, such as HSV, and

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Figure 5: Complete regression of the lesions with normal mucosa at the 1 month follow-up

(3) a history of the use of new medication. Clinical clues to diagnosis comprise the presence of target lesions, raised atypical papules or mucosal involvement, or a combination of these.[2] Biopsies are helpful only in the early vesicular lesions and not in the ulcerated as histopathologic appearances are nonspecific. Immunostaining shows intense lymphocytic infiltration at the basement membrane zone and perivascularly, nonspecific immune deposits of IgM, C3, and fibrin at these sites.[4] Management initiates with identification of triggering agent, if precipitant is drug (commenced in the last 7–21 days is the likely culprit) defer it. Considering the viral etiology, in a majority of cases acyclovir 400 mg q.i.d. for a period of 6 months or continuous treatment using valacyclovir 500 mg b.i.d. is given as a prophylaxis. Palliative therapy such as topical analgesics, viscous lidocaine rinses, soothening mouth rinses, along with bland soft diet, avoidance of acidic, and spicy food.[9] The mainstay of treatment is topical and systemic corticosteroids with a tapering protocol.[4] Azathioprine, cyclophosphamide, dapsone, cyclosporine, levamisole, thalidomide, interferon‑α, and cyclosporine are drugs of choice in recalcitrant cases.[2]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published

Figure 6: Dermal lesions completely regressed

and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship Nil.

Conflicts of interest

There are no conflicts of interest.

References 1. Sen P, Chua SH. A case of recurrent erythema multiforme and its therapeutic complications. Ann Acad Med Singapore 2004;33:793‑6. 2. Simbli MA. Erythema multiforme: Challenging diagnosis for internist. J Clin Case Rep 2013;3:285‑8. 3. 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. 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 2010;16:145‑8. 5. Gantala R, Devaraju RR, Goud S, Aitha H, Kubbi JR. Condiments costing high...! A case report of erythema multiforme. J Clin Diagn Res 2015;9:ZD22. 6. Williams PM, Conklin RJ. Erythema multiforme: A review and contrast from Stevens‑Johnson syndrome/toxic epidermal necrolysis. Dent Clin N Am 2005;49:67‑76. 7. Satpute P, Yadav L, Ahmed R, Kashid A, Peter K. Herbal tooth powder induced erythema multiforme. J Clin Diagn Res 2014;8:275‑6. 8. Kohli PS, Kaur J. Erythema multiforme‑oral variant: Case report and review of literature. Indian J Otolaryngol Head Neck Surg 2011;63:9‑12. 9. Bharati P, Sushmini H, Shobha N, Rakesh S. Oral blistering ‑ Report of two cases of erythema multiforme & literature review. J Clin Diagn Res 2013;7:2080‑3.

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