A major cause of erythema multiforme is the herpes virus hsv. Characterised by target lesions that resemble a bulls eye. Erythema multiforme em is an acute, immunemediated disorder affecting the skin andor mucous membranes, including the oral cavity. Firoozeh samim, dmd, msc a, ajit auluck, bds, mds, phd b. Hsvviral dna fragments are transported by peripheral blood mononuclear cells to distant skin sites. Erythema bullosa stevensjohnson syndrome1 pathogenesis acth. Clinical features include a rash of varied appearance, beginning as. Clinical features, diagnosis, and treatment of erythema. Glioblastoma multiforme gbm accounts for more than half of mg cases. Erythema multiforme em is a clinical conundrum, the name of which reflects the broad morphological spectrum of the lesions 1. It causes a rash of spots with dark centers and pale red rings that look like a target or bullseye.
Typically presents in a symmetrical distribution of. Erythema multiforme is a skin condition considered to be a hypersensitivity reaction to infections or drugs. Erythema multiforme for parents nemours kidshealth. Erythema multiforme bullosa stevens johnson syndrome his condition on admission is seen in the accompanying photograph. Understanding the pathogenesis of hsvassociated erythema. Clinical features, diagnosis, and treatment of erythema multiforme. Clinical features a concentric ring like appearance of the lesions, resulting from the varying shades of erythema, occurs in some cases and has given rise to the terms target, iris, or bulls eye most common on the hands, wrists and ankles. The main mechanisms describing the pathogenesis of em have been based on. Mucosal changes, if present, consist initially of redness of the lips and inside the cheek.
Dec 01, 2006 erythema multiforme usually occurs in adults 20 to 40 years of age,6 although it can occur in patients of all ages. Department of oral pathology and microbiology, bhojia dental college and hospital, solan. Erythema multiforme em is a rare skin disorder that mainly affects children. It is an uncommon disorder, with peak incidence in the second and third decades of. Pdf etiopathogenesis of erythema multiforme a concise. It is characterized by skin eruptions, with or without oral or other mucus membrane lesions. Feb 20, 2020 erythema multiforme em is an acute, selflimited, and sometimes recurring skin condition that is considered to be a type iv hypersensitivity reaction associated with certain infections, medications, and other various triggers.
Erythema multiforme em is an acute, immunemediated disorder affecting the skin andor mucous membranes, including the. Ferril has also written various features for medical newsletters. Ordered treatment plans will be sent immediately electronically as pdf documents and come with a 30day guarantee if you feel, for any reason, that the educational information given does not contribute to helping your particular issue. After 14 days of treatment, skin and oral lesions were controlled. Erythema multiforme an overview sciencedirect topics. Erythema multiforme em is an ulcerative, blistering inflammatory condition which affects either the skin or the mucous membranes, most often manifesting as a mucocutaneous lesion. Pathogenesis of erythema multiforme is a frequently mentioned supplication for the reason that it involves erythema migrans differential, eritema nodosum slike, and erythema migrans differentialdiagnose. The pathophysiology of erythema multiforme em is still not completely understood, but it is probably immunologically mediated and appears to involve a hypersensitivity reaction that can be triggered by a variety of stimuli, particularly. Pathogenesis genetic susceptibility can be a predisposing factor in some patients with em. A case of erythema multiforme was described in a 9yearold boy following treatment with sertraline.
The disorder is most commonly induced by infection, with herpes simplex virus being the most frequent precipitator. Erythema multiforme may be present within a wide spectrum of severity. Em is mainly seen in adults between 2040 years of age. The remainder of this article will focus on erythema multiforme. Etiology and pathophysiology erythema multiforme usually occurs in adults 20 to 40 years of age,6 although it can occur. Erythema multiforme em is a clinical conundrum, the name. Erythema multiforme adverse skin reactions to ssris are known to include rashes, erythema multiforme, stevenjohnson syndrome and toxic epidermal necrolysis 52 r, 53 r. A mild, selflimiting, potentially recurring mucocutaneous inflammatory condition.
These usually erupt over 24 to 48 hours and last for 1 to 2 weeks. It is an acute, selflimited, episodic and mucocutaneous inflammatory disorder of the skin and mucous membranes that could be related to immunologic 2 hypersensitivity reaction to certain infections, medications, and other various triggers 3. Mucosal lesions, if present, typically develop a few days after the skin rash begins in erythema multiforme minor, mucous membrane involvement is absent or mild. Erythema multiforme stevensjohnson syndrome, erythema multiforme major, erythema multiforme minor, herpesinduced em major, herpesassociated erythema multiforme, druginduced stevensjohnson syndrome. Somespeculation onthe mechanismofabsorption of the vesicle fluid may be profitable of stevensjohnson syndrome. It consists of a polymorphous eruption of macules, papules, and characteristic target lesions that are symmetrically distributed with a propensity for the distal extremities. The term erythema multiforme em includes a wide range of expressions, from exclusive oral erosions oral em to mucocutaneous lesions ranging from mild em minor to severe multiple mucosal membranes em major, stevensjohnson syndrome sjs or large area of total body surface toxic epidermal necrolysis ten. Erythema may arise from a great variety of causes and disease conditions. Management of erythema multiforme associated with recurrent. Erythema multiforme is characterized by the sudden eruption of crops of red or violet flat spots, wheals, papules small solid. The disease is usually related to an acute infection, most often a recurrent herpes simplex virus hsv infection. Day skin cancer and immunodermatology laboratories, memorial sloankettering cancer center, new york, nem york 10021 received. They are associated with high morbidity and mortality.
A variety of factors have been implicated in the pathogenesis of em. A study revealed that the cutaneous lesions of patients with haem were infected with hsv1 in 66. Erythema multiforme em is a rare, acute hypersensitivity reaction most commonly triggered by herpes simplex virus hsv infections. Clinical immunology and immunopathology 7, 379385 1977 erythema multiforme. Expression of hsv dna in keratinocytes leads to activation of hsvspecific cd41 th1 cells, which produces interferon g. Apoptotic individual keratinocytes cellular selfdestruction, earliest histological change hydropic degeneration of basal keratinocytes swollen degenerating cells at the base of the epidermis intercellular oedema spongiosis.
It often also affects the mouth, eyes and other moist surfaces. A skin biopsy of erythema multiforme em may show in the epidermis epithelium. Understanding the pathogenesis of hsvassociated em haem is essential for patient management. Jul 12, 2012 erythema multiforme em is an acute, immune. Epidemiology, pathogenesis, symptomatology, diagnosis, and management mahnaz fatahzadeh, dmd,a and robert a. Erythema multiforme introduction and epidemiology erythema multiforme em is an acute, immunemediated condition, most commonly induced by herpes simplex virus hsv infection, or by the use of medications, such as phenytoin, sulfonamides, penicillins, and barbiturates.
The definition of em in this chapter is based on the classification proposed by bastujigarin et al. Herpesassociated erythema multiforme haem appears to represent the result. Erythema multiforme oral lesions is a logical request due to the reason that it is applicable to erythema migrans lesions, erythema migrans rash, and erythema multiforme a. It is an acute, selflimited, episodic and mucocutaneous inflammatory disorder of the skin and mucous membranes that could be related to immunologic 2 hypersensitivity reaction to certain infections, medications, and other various. Erythema multiforme em is an acute, immunemediated condition, most commonly induced by herpes simplex virus hsv infection, or by the use of medications, such as phenytoin, sulfonamides, penicillins, and barbiturates. Various explanations for this cutaneous eruption have been given and include the following. Erythema multiforme is a reactive mucocutaneous disorder in a disease spectrum that comprises a selflimited, mild, exanthematic, cutaneous variant with minimal oral involvement em minor to a progressive, fulminating, severe variant with extensive mucocutaneous epithelial necrosis sjs.
Erythema multiforme bullosa stevens johnson syndrome although they are of value in the presence of secondary infection. Erythema gyratum repens egr is a figurate erythema that is believed to be a paraneoplastic process. Em with mucosal involvement is known as erythema multiforme major, and. Erythema multiforme epidemiology bmj best practice.
Target or iris lesions distributed symmetrically on the extremities and trunk characterize the condition. In fact, recent or recurrent herpes has been reported as the principle risk factor for erythema multiforme. Clinical and histologic features led us to the diagnosis of erythema multiforme. Erythema multiforme em is an acute selflimited, usually mild, and often relapsing mucocutaneous syndrome. What is the role of hsv in the pathogenesis of erythema. Erythema multiforme em is an acute mucocutaneous hypersensitivity reaction with a variety of etiologies. Such unusual forms of fde may be easily overlooked unless clinicians take special care to recognize the presence of such variants 17. When seen in adults, it usually occurs between the ages of. In erythema multiforme minor, mucous membrane involvement is absent or mild. Erythema multiforme has different forms, such as erythema multiforme minor, which is not very serious when compared to erythema multiforme major stevensjohnson syndrome, a more severe and sometimes fatal condition. Multiforme polymorphic first classified by bastuji garin et al to separate from sjs ten in 1922 described by hebra in 1866 as erythema exudativum multiforme 122920. Erythema multiforme is a distinctive hypersensitivity syndrome characterized by skin and mucous membrane lesions and, in its more severe forms, mucosal lesions with constitutional symptoms and, at times, visceral involvement. Abstract erythema multiforme em is an uncommon, immune.
Palmar erythema pe, an often overlooked physical finding, is due to several physiologic or systemic pathologic states. Pathogenesis and treatment, pharmacology and therapeutics 2015, doi. Erythema multiforme knowledge for medical students and. Depending on the type of erythema, the cause will vary. Erythema multiforme has been so named because of the multiple forms it. Erythema multiforme ppt 1 free ebook download as powerpoint presentation. Erythema multiforme airuhtheemuh multehformee starts with pink or red blotches. Other associated infections include hepatitis b, epsteinbarr virus, cytomegalovirus, histoplasmosis with concomitant erythema nodosum, orf a disease of sheep and goats caused by a parapox virus that can be transmitted to humans, coccidioidomycosis, kawasaki disease, eun ly, go jw, kang wh, et al. Report of two cases and speculation on immune mechanisms involved in the pathogenesis bijan safai, robert a.
Immune complexes in the sera and in blister fluids of patients with erythema multiforme have been detected by the. The rash is made of spots that are red welts, sometimes with purple or blistered areas in the center. In addition to other features, characteristic concentric erythematous bands forming a woodgrain appearance help distinguish erythema gyratum repens from other figurate erythemas, such as erythema annulare centrifugum, erythema migrans, and e. The most common etiologic association with erythema multiforme is herpes simplex virus hsv infection, which is frequently concomitant with the em flare. Erythema multiforme em is a polymorphic, often recurring eruption caused by exposure to medication or various infections, notably herpes simplex virus hsv. In erythema multiforme major, one or more mucous membranes are typically affected, most often the oral. Erythema multiforme introduction and epidemiology etiology. From a medical point of view, the two major erythemas are erythema multiforme and erythema nodosum. With hsv associated erythema multiforme the pathogenesis is felt to be a delayedtype hypersensitivity reaction.
This also affects the symptoms the individual will suffer from. Each year, about 56 cases out of 100,000 people are diagnosed with primary malignant brain tumors, of which about 80% are malignant gliomas mgs. Erythema multiforme em is an acute, selflimited, and sometimes recurring skin condition that is considered to be a type iv hypersensitivity reaction associated with certain infections, medications, and other various triggers. See more images of erythema multiforme mucous membrane involvement. We present a case of a woman with a 14year history of rheumatoid arthritis, who showed simultaneously and gradually appearing, annular, erythematous, itchy patches and exacerbation of the joint symptoms, of one month duration, after pregnancy. The pathophysiology of erythema multiforme em is still not completely understood, but it is probably immunologically mediated and appears to involve a hypersensitivity reaction that can be triggered by a variety of stimuli, particularly bacterial, viral. Erythema multiforme em is a skin condition of unknown cause. Some spots can have a small blister or scab in the middle. His eyes were closed with purulent exudate and oedemabut the conjuctivae and cornae were clear. Considerable attention has been directed in recent years to the etiology of erythema nodosum. Erythema multiforme em is an acute, immunemediated condition characterized by the appearance of distinctive targetlike lesions on the skin.
We describe a 52yearold man who was diagnosed with chronic hepatitis c, preceding the appearance of lichen planus, erythema nodosum, and erythema multiforme that coincided with the reactivation of viral replication. The pathophysiology of erythema multiforme em is still not completely understood, but it is probably immunologically mediated and appears. Epidermal cell damage results from attacks by cytotoxic tcells, nk cells, and monocytes. Schwartz, md, mphb newark, new jersey eight of the more than 80 known herpesviruses are human pathogens. A blotchy maculopapularrashwaspresent onthe face, trunk. Etiology and pathophysiology of stevensjohnson syndrome clinical presentation of stevensjohnson syndrome in children diagnostic workup for stevensjohnson syndrome in children. The role of systemic corticosteroid therapy in erythema.
Interaction of hsv1 infected peripheral blood mononuclear. Erythema multiforme minor refers to em without or with only mild mucosal disease and without associated systemic symptoms. Herpes associated erythema multiformea diagnostic dilemma. Additional and relevant useful information for erythema multiforme. Lichen planus, erythema nodosum, and erythema multiforme in a. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Sometimes blisters develop and quickly break to form erosions and ulcers. What is the pathophysiology of erythema multiforme em.