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Number IV Erythema multiforme

Profile image of Jose BaganJose Bagan

2005, Oral Diseases

Abstract

Erythema multiforme (EM) is an acute mucocutaneous hypersensitivity reaction characterised by a skin eruption, with or without oral or other mucous membrane lesions. Occasionally EM may involve the mouth alone. EM has been classified into a number of different variants based on the degree of mucosal involvement and the nature and distribution of the skin lesions. EM minor typically affects no more than one mucosa, is the most common form and may be associated with symmetrical target lesions on the extremities. EM major is more severe, typically involving two or more mucous membranes with more variable skin involvement-which is used to distinguish it from Stevens-Johnson syndrome (SJS), where there is extensive skin involvement and significant morbidity and a mortality rate of 5-15%. Both EM major and SJS can involve internal organs and typically are associated with systemic symptoms. Toxic epidermal necrolysis (TEN) may be a severe manifestation of EM, but some experts regard it as a discrete disease. EM can be triggered by a number of factors, but the best documented is preceding infection with herpes simplex virus (HSV), the lesions resulting from a cell mediated immune reaction triggered by HSV-DNA. SJS and TEN are usually initiated by drugs, and the tissue damage is mediated by soluble factors including Fas and FasL.

Figures (4)
Figure 2 Pathogenesis of EM HAEM it is most likely that HSV-DNA fragments in the skin or mucosa precipitate the disease. HSV-DNA fragments and in particular DNA polymerase (PoL) have been detected in the basal and suprabasal cell layers of the epidermis in lesions as well as healed lesions for up to 3 months (Imafuku et al, 1997) and the T cells accumulating in active lesions are CD4+ (Vf$2+) cells which respond to HSV antigens in vitro (Malmstrom et al, 1990; Kokuba et al, 1998). In addition there is a good correlation between PoL expression in lesions, CD4+ (Vf2+) T cell accumulation and the duration of clinical symptoms (Kokuba et al, 1998). In situ RT-PCR and immunocytochemical staining have shown that mononuclear cells in both HAEM and HSV lesional skin tissues stain positively for IFN-y, a cytokine, which is associated with tissue damage, and this expression correlates with HSV-protein expression (Kokuba et al, 1999). IFN-y is produced by CD4+ T helper | (Th1l) lymphocytes, cells characteristic of a delayed type hypersensitivity reaction (DTH) (Billiau et a/, 1998) (Figure 2). IFN-y is pro-inflammatory and induces
Figure 2 Pathogenesis of EM HAEM it is most likely that HSV-DNA fragments in the skin or mucosa precipitate the disease. HSV-DNA fragments and in particular DNA polymerase (PoL) have been detected in the basal and suprabasal cell layers of the epidermis in lesions as well as healed lesions for up to 3 months (Imafuku et al, 1997) and the T cells accumulating in active lesions are CD4+ (Vf$2+) cells which respond to HSV antigens in vitro (Malmstrom et al, 1990; Kokuba et al, 1998). In addition there is a good correlation between PoL expression in lesions, CD4+ (Vf2+) T cell accumulation and the duration of clinical symptoms (Kokuba et al, 1998). In situ RT-PCR and immunocytochemical staining have shown that mononuclear cells in both HAEM and HSV lesional skin tissues stain positively for IFN-y, a cytokine, which is associated with tissue damage, and this expression correlates with HSV-protein expression (Kokuba et al, 1999). IFN-y is produced by CD4+ T helper | (Th1l) lymphocytes, cells characteristic of a delayed type hypersensitivity reaction (DTH) (Billiau et a/, 1998) (Figure 2). IFN-y is pro-inflammatory and induces
Figure 1 Histopathology of EM minor. The epithelium is oedematous and intra- and sub-epithelial vesicles are present. An infiltration of lymphocytes and macrophages is seen in the lamina propria and within the epithelium
Figure 1 Histopathology of EM minor. The epithelium is oedematous and intra- and sub-epithelial vesicles are present. An infiltration of lymphocytes and macrophages is seen in the lamina propria and within the epithelium
Figure 3 Clinical appearance of EM Skin lesions have been classified as ‘typical targets’, ‘raised atypical targets’, ‘flat atypical targets’ and ‘erythe- matous macules with or without blisters’ (Bastuji-Garin et al, 1993) and these have been used to sub-classify EM (see below). “Typical targets’ are defined as ‘individual lesions less than 3 cm diameter with a regular round shape, a well-defined border, and two concentric
Figure 3 Clinical appearance of EM Skin lesions have been classified as ‘typical targets’, ‘raised atypical targets’, ‘flat atypical targets’ and ‘erythe- matous macules with or without blisters’ (Bastuji-Garin et al, 1993) and these have been used to sub-classify EM (see below). “Typical targets’ are defined as ‘individual lesions less than 3 cm diameter with a regular round shape, a well-defined border, and two concentric
Table 1 Drug-related EM (SJS and TEN)
Table 1 Drug-related EM (SJS and TEN)

Related papers

Erythema Multiforme Major: Case Report
Kedar Saraf

International Journal of Oral and Maxillofacial Pathology, 2012

Erythema Multiforme is an acute inflammatory disease of the skin and mucous membranes that causes a variety of skin lesions – hence the name "multiforme". It is a blistering, ulcerative condition of uncertain etiopathogenesis. Hall mark of this is the iris or target lesion. EM may present within a wide spectrum of severity. Among the many etiologic factors the most common triggers for episodes of EM are herpes simplex virus and drug reactions. Drugs are reported in many documented cases of Stevens-Johnson syndrome and Toxic Epidermal Necrolysis. Sulfa drugs are the most common triggers. Here is the case of erythema multiforme major secondary to drug reaction with oral, skin and genital manifestations.

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Erythema multiforme: diagnosis, clinical manifestations and treatment in a retrospective study of 22 patients
Jose Sanchis

Journal of Oral Pathology & Medicine, 2010

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Erythema multiforme and related disorders
Stefano Fedele

Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology, 2007

Erythema multiforme (EM) and related disorders comprise a group of mucocutaneous disorders characterized by variable degrees of mucosal and cutaneous blistering and ulceration that occasionally can give rise to systemic upset and possibly compromise life. The clinical classification of these disorders has often been variable, thus making definitive diagnosis sometimes difficult. Despite being often caused by, or at least associated with, infection or drug therapy, the pathogenic mechanisms of these disorders remain unclear, and as a consequence, there are no evidencebased, reliably effective therapies. The present article reviews aspects of EM and related disorders of relevance to oral medicine clinical practice and highlights the associated potential etiologic agents, pathogenic mechanisms and therapies.

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Erythema Multiforme and Stevens Johnson syndrome: different spectrum of an entity or different entities?
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EM and SJS are diverse disorders, differing significantly in connotation, rash characteristics, clinical course, treatment strategies, and overall outcome.

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Sathawane et. al : Erythema Multiforme Major : Case Report and Review ERYTHEMA MULTIFORME MAJOR: CASE REPORT AND REVIEW OF LITERATURE ADDRESS FOR CORRESPONDENCE
Samiksha Tripathi

2016

Erythema multiforme is an acute mucocutaneous disorder that occurs with varying degrees of blistering and ulceration. We report a case of major erythema multiforme managed with systemic steroids. A 45-year-old male had cutaneous target lesions and ulcerative lesions throughout the oral cavity and lips, which had been diagnosed as erythema multiforme major. This episode was related to neither drug intake nor herpetic infection, which suggests that the erythema multiforme was of idiopathic origin. This hypothesis was supported by negative serology for herpes simplex virus. Excisional biopsy of an intact bulla was performed and the diagnosis was confirmed as erythema multiforme major. The patient was treated with Prednisolone in a tapering dose for 2 months to control and completely cure the disease.

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