|Year : 2017 | Volume
| Issue : 2 | Page : 153-155
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
|Date of Submission||27-Dec-2016|
|Date of Acceptance||24-Oct-2017|
|Date of Web Publication||9-Nov-2017|
Department of Oral Medicine and Radiology, Sri Venkata Sai Institute of Dental Sciences, Appanapally, Mahabubnagar, Telangana
Source of Support: None, Conflict of Interest: None
| 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
|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
| 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. EM is triggered primarily by the antigens, which are induced by exposure to microbes or by certain medication. Viral etiology is the most common.
| 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 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.
|Figure 1: Series of pictures depicting multiple encrustations, and ulcerations, with clinically evident bleeding|
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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 [Figure 3] and [Figure 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 instructions were given. Total resolution of the oral and dermal lesions was attained after 1 month [Figure 5] and [Figure 6]. The patient is currently under review with no recurrence at 1 year follow-up.
|Figure 3: Series of intraoral photographs showing partially regressed lesions after 1 week under medication|
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|Figure 5: Complete regression of the lesions with normal mucosa at the 1 month follow-up|
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| Discussion|| |
It dates back to 1866 where Von Hebra first mentioned the acral distribution of target iris lesions in EM with or without mucosal involvement. 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. 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), which is drawn in up to 70–80% of the cases.,
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 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. On the contrary, drug-associated EM seems to involve CD8+ T-cell attack and expression of TNF-α in the lesion.
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.,, 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.
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 (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. 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.
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. The mainstay of treatment is topical and systemic corticosteroids with a tapering protocol. Azathioprine, cyclophosphamide, dapsone, cyclosporine, levamisole, thalidomide, interferon-α, and cyclosporine are drugs of choice in recalcitrant cases.
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 and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]