Mar 27, 2019 erythema gyratum repens is associated with malignancy in as many as 80% of patients. Erythema multiforme em is an acute, selflimited skin condition. Erythema multiforme em is an acute, immunemediated condition, most commonly induced by herpes simplex virus hsv infection, or by the use of certain medication and presents with cutaneous or. His eyes were closed with purulent exudate and oedemabut the conjuctivae and cornae were clear. Erythema multiforme is a hypersensitivity reaction usually triggered by infections, most commonly herpes simplex virus hsv. Erythema bullosa stevensjohnson syndrome1 pathogenesis acth.
Erythema multiforme for parents nemours kidshealth. Erythema multiforme, stevensjohnson syndrome and toxic. Despite multiple associations, thought to be triggered by hsv in most cases. The pathogenesis of herpesassociated erythema multiforme has been well studied and is. Erythema multiforme may be present within a wide spectrum of severity. The ocular picture varied from mild mucopurulent conjunctivitis to perforating corneal ulcers destroying the globe. Erythema multiforme classification and immunopathogenesis. Erythema multiforme minor refers to em without or with only mild mucosal disease and without associated systemic symptoms. The most common etiologic association with erythema multiforme is herpes simplex virus hsv infection, which is frequently concomitant with the em flare. However, since that time, erythema multiforme major has been most commonly referred to as stevensjohnson syndrome.
Clinical features, diagnosis, and treatment of erythema multiforme. Erythema multiforme em is an acute, selflimited, inflammatory cutaneous disorder caused by infection or drugs that is characterized by bulls eye lesions, blisters, and erythematous plaques on the skin fitzpatrick and bernhard 1987. Erythema multiforme em is an acute, immunemediated condition characterized by the appearance of distinctive targetlike lesions on the skin. 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 and. Granular c3 and igm at basement membrane and in vessels. The exact pathogenesis of erythema multiforme is not known. It is an uncommon disorder, with peak incidence in.
Their coexistence is known as rowell syndrome rs, first described in 1963. Stevensjohnson syndrome and toxic epidermal necrolysis are severity variants of the same disease which differs from erythema multiforme. A variety of factors have been implicated in the pathogenesis of em. 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. We report a case of recurrent herpesassociated erythema multiforme managed with prophylactic acyclovir. Erythema multiforme is a skin reaction that can be triggered by an infection or some medicines. Erythema multiforme em and systemic lupus erythematosus sle are common diseases. Erythema multiforme em is a clinical conundrum, the name. 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. Erythema multiforme major is the term used to describe em with severe mucosal involvement and. Erythema multiforme mainly affects adults under 40. 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. 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.
Hsvviral dna fragments are transported by peripheral blood mononuclear cells to distant skin sites. The pathogenesis of herpesassociated erythema multiforme is consistent with a delayedtype hypersensitivity reaction. Erythema multiforme an overview sciencedirect topics. Erythema gyratum repens has also been associated with anal cancer. An 11yearold boy had lesions in the oral cavity and lips, which had been diagnosed as erythema multiforme. Theres also a rare, severe form that can affect the mouth, genitals and eyes and can be lifethreatening. These lesions are often accompanied by erosions or bullae involving the oral, genital, andor ocular mucosae picture 2ac. Erythema multiforme introduction and epidemiology etiology. In half of the cases, the triggering agents appear to be medications, including anticonvulsants. They are associated with high morbidity and mortality.
Erythema multiforme is a skin condition considered to be a hypersensitivity reaction to infections or drugs. Ferrils treatment plans are designed to be shared with other providers as educational advice only. A skin biopsy of erythema multiforme em may show in the epidermis epithelium. Erythema multiforme, especially em major and sjs can occur after.
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. Erythema multiforme bullosa stevens johnson syndrome his condition on admission is seen in the accompanying photograph. Somespeculation onthe mechanismofabsorption of the vesicle fluid may be profitable of stevensjohnson syndrome. Mucous membranes are affected in over 90 percent of patients, usually at two or more distinct sites ocular, oral, and genital. In 1950, thomas suggested the division of erythema multiforme into two forms. 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. Erythema multiforme em refers to a group of hypersensitivity disorders characterized by symmetric red, patchy lesions, primarily on the arms and legs. The disorder is most commonly induced by infection, with herpes simplex virus being the most frequent precipitator. The most commonly reported malignancies with pnp are lymphoid malignancies, most often non hodgkin lymphoma and chronic lymphocytic leukemia, followed by castleman disease, thymoma, and a mix of other solid organ tumors. When involvement of the mucous membrane accompanies erythema multiforme bullosum, serious ocular sequelae may occur. The cause is unknown, but em frequently occurs in association with herpes simplex virus, suggesting an immunologic process. Sanders dy, johnson he 1971 stevensjohnson syndrome associated with mycoplasma pneumoniae infection. Mucous membrane lesions without cutaneous involvement em is usually called minor when mucous membranes are spared or minimally affected, for example, lips, and majus or major when at least two mucosal sites are involved.
Classified as erythema multiforme minor or erythema. Erythema multiforme em is an acute, immunemediated condition characterized by the appearance of distinctive targetlike lesions on the skin picture 1ah. Despite current multimodality treatment efforts including maximal surgical resection if feasible, followed by a combination of radiotherapy andor chemotherapy, the median survival is short. In 1922, two american pediatricians, stevens and johnson, described two boys with a more. Its histopathology is characterized by dense infiltrates of t lymphocytes 14. Drugs adverse skin reactions to drugs are frequent, with rates of reaction to many commonly used drugs exceeding 1% 14. Erythema multiforme em is an acute mucocutaneous hypersensitivity reaction with a variety of etiologies. Erythema multiforme em is an acute mucocutaneous condition caused by a hypersensitivity reaction. Erythema multiforme, especially em major and sjs can occur after taking certain medications, although medications are a less likely cause than an infection 15. Europe pmc is an archive of life sciences journal literature.
Erythema multiforme nord national organization for rare. Herpes associated erythema multiformea diagnostic dilemma. Not all cases of erythema multiforme em can be prevented, especially if the offending agent is not apparent or remains unknown. It is acute and selflimiting, usually resolving without complications. Viral skin diseases picture of erythema multiforme minor erythema multiforme em minor is a bullseye rash with alternating rings of red erythema and flaky skin desquamation. Stevensjohnson syndrome and toxic epidermal necrolysis. Erythema gyratum repens is associated with malignancy in as many as 80% of patients. Erythema multiforme em is the name applied to a group of hypersensitivity disorders, affecting mostly children and young adults, and characterized by symmetric red, patchy lesions, primarily on the arms and legs. Erythema multiforme stevensjohnson syndrome, erythema multiforme major, erythema multiforme minor, herpesinduced em major, herpesassociated erythema multiforme, druginduced stevensjohnson syndrome 3. Management of erythema multiforme associated with recurrent.
Pathogenesis of erythema multiforme is a commonly identified supplication in light of the fact that it is of interest when evaluating erythema multiforme lips, erythema multiforme mouth, and erythema multiforme prednisone. Mucous membrane lesions without cutaneous involvement em is usually called minor when mucous membranes are spared or minimally affected, for example, lips, and majus or major when at. Clinical features, diagnosis, and treatment of erythema. Erythema multiforme is an acute mucocutaneous disorder, characterized by varying degrees of blistering and ulceration.
It is characterized by skin eruptions, with or without oral or other mucus membrane lesions. Em minor, a mild allergic reaction, occurs most commonly. These usually erupt over 24 to 48 hours and last for 1 to 2 weeks. Erythema multiforme bullosa stevens johnson syndrome. With hsv associated erythema multiforme the pathogenesis is felt to be a delayedtype hypersensitivity reaction. Erythema multiforme bullosa stevensjohnson syndrome. 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. Some spots can have a small blister or scab in the middle. Of 162 previously reported cases of erythema multiforme with mucosal involvement, the eyes were affected in 148. Erythema multiforme em is a skin condition of unknown cause. Epidemiology of erythema exsudativum multiforme majus, stevensjohnson syndrome, and toxic epidermal necrolysis in germany 1990.
A study revealed that the cutaneous lesions of patients with haem were infected with hsv1 in 66. 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. Nov 14, 2011 erythema multiforme em refers to a group of hypersensitivity disorders characterized by symmetric red, patchy lesions, primarily on the arms and legs. Erythema multiforme bullosa stevens johnson syndrome although they are of value in the presence of secondary infection. Among visceral malignancies, the lung is the most common site, 7, 8, 9 followed by the breast, urinary bladder, uterus andor cervix, gi tract stomach, and prostate. The cause is unknown, but em frequently occurs in association with herpes simplex virus, suggesting an immunologic process initiated by the virus. Erythema multiforme em is a clinical conundrum, the name of which reflects the. Pathogenesis and treatment, pharmacology and therapeutics 2015, doi. The role of systemic corticosteroid therapy in erythema.
We present two cases of sle in the setting of a newly developed emlike eruption, which shares many similarities with the socalled rowell syndrome. It presents with a skin eruption characterised by a typical target lesion. It is an uncommon disorder, with peak incidence in the second and third decades of life. Systemic lupus erythematosus associated with erythema. Erythema multiforme airuhtheemuh multehformee starts with pink or red blotches. Most patients with erythema gyratum repens develop the eruption. Erythema multiforme em is an acute, immunemediated condition, most commonly induced by herpes simplex virus hsv infection, or by the use of.
Erythema multiforme dental clinics of north america. Glioblastoma multiforme gbm accounts for more than half of mg cases. Considerable attention has been directed in recent years to the etiology of erythema nodosum. A blotchy maculopapularrashwaspresent onthe face, trunk andlimbs. Only few cases of rs have been described and some of them questioned its existence. Typically presents in a symmetrical distribution of lesions over the dorsal surfaces of. Erythema multiforme major is the term used to describe em with severe mucosal involvement and may. A mild, selflimited, potentially recurring mucocutaneous inflammatory condition. The pathophysiology of erythema multiforme em is still not completely understood, but it is probably immunologically mediated and appears. Various explanations for this cutaneous eruption have been given and include the following.
Characterized by target lesions that resemble a bulls eye. Erythema multiforme genetic and rare diseases information. Despite being listed with many etiological factors, the exact pathogenic mechanism of em remains unclear, and as a consequence, there are no evidencebased. Etiology and pathophysiology erythema multiforme usually occurs in adults 20 to 40 years of age,6 although it can occur. Stevensjohnson syndrome sjs and toxic epidermal necrolysis ten are severe mucocutaneous reactions, most commonly triggered by medications, characterized by extensive necrosis and detachment of the epidermis 1. Etiopathogenesis of erythema multiforme juniper publishers. Firoozeh samim, dmd, msc a, ajit auluck, bds, mds, phd b. Outcomesresolutions most erythema multiforme minor skin lesions often resolve in about 26 weeks. Mucosal erythema multiforme fuchs syndrome, ectodermosis pluriorificialis. Peak incidence in second and third decades of life.1029 622 1001 1222 678 547 732 646 494 452 597 1391 737 1278 783 131 1489 423 172 1457 1057 920 1173 1520 837 1380 1014 1425 421 1428 639 1352 868 140 871 883 519 1228 835 122 859 341 1320 499 1361 357