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Erythema Multiforme
Erythaema Polymorphe

Erythema multiforme is a rare skin condition characterized by targetoid lesions.

Images

WIKIDATA, CC BY-SA 3.0
WIKIDATA, CC BY-SA 3.0
WIKIDATA, CC BY-SA 3.0

Presentation

The most common sign of EM is the presence of a target lesions, although they may not always be present and variable mucous membrane involvement [7]. EM minor presents as a mild to severe rash consisting of erythematous papules that are usually ranged symmetrically on the extremities and gradually enlarge. The typical lesions can develop a raised, edematous, pale ring and the outer edges will become a cyanotic or violaceous halo. After the inflammation resolves, hyperpigmentation or hypopigmentation can occur. Some affected individuals may experience pruritus and a burning sensation.

Entire Body System

  • Fever

    Syphilis, erythema multiforme, fever and rash, fever of unknown origin Syphilis is a sexually transmitted disease known to present with highly variable manifestations, especially when left untreated. [ejcrim.com]

    The acute disease is often associated with fever, malaise, and arthralgias. [medical-dictionary.thefreedictionary.com]

    Blood test performed in 4th day of fever confirmed the picture of systemic inflammation (Table 1). In 6th day of fever the child showed mild bilateral bulbar conjunctival injection without exudate. [ijponline.biomedcentral.com]

    EM major often starts with a fever and joint aches. Besides the skin sores and mouth sores, there may be sores in the eyes, genitals, lung airways, or gut. Your health care provider will look at your skin to diagnose EM. [nlm.nih.gov]

    Mild symptoms including feeling unwell, low-grade fevers, sore joints or coughing may precede the classic rash. Early on, itching and burning in the skin may be present. In some cases there may be no preceding symptoms. [dermcoll.edu.au]

  • Inflammation

    In the absence of classical presentation, the laboratoristic evaluation of systemic inflammation can help in placing the correct diagnosis to promptly start adequate therapy. [ncbi.nlm.nih.gov]

    Histologically, each shows varying degrees of dermal and epidermal inflammation and injury. The clinical manifestations are determined by both the intensity of the damage and the relative amount of injury to each layer. [link.springer.com]

    In severe cases, possible complications can include: sepsis a skin infection (cellulitis) permanent skin damage and scarring permanent eye damage inflammation of internal organs, such as the lungs or liver Page last reviewed: 13 September 2019 Next review [nhs.uk]

  • Swelling

    A 15-year-old girl presented with recurrent lip swelling and eventual development of diarrhea and targetoid macules on the palms, feet, and back. [ncbi.nlm.nih.gov]

    Large hives often are accompanied by swelling of the hands and feet; but, Erythema Multiforme is not accompanied by such swelling. [tlcpediatrics4u.com]

    Mucosal lesions consist of swelling and redness with blister formation. The blisters break quickly to leave large, shallow, irregular shaped, painful ulcers that are covered by a whitish pseudomembrane. [dermnetnz.org]

    Cases with mucosal involvement typically consist of redness in the affected area, which may develop erosions or ulcerations and are accompanied by swelling and pain. [ozarkderm.com]

  • Fatigue

    Adverse effects, observed in 4 patients, included fatigue, macrocytic anemia, anxiety, insomnia and involuntary movements, and drug-induced lupus erythematosus. [ncbi.nlm.nih.gov]

    The following are the most common symptoms of erythema multiforme: Sudden, red patches and blisters, usually on the palms of hands, soles of feet, and face Flat, round red "targets" (dark circles with purple-grey centers) Itching Cold sores Fatigue Joint [hopkinsmedicine.org]

  • Asymptomatic

    She was asymptomatic, had no skin lesions, and received a prescription for nivolumab. [elsevier.es]

    Symptoms and Signs of Erythema Multiforme Erythema multiforme manifests as the sudden onset of asymptomatic, erythematous macules, papules, wheals, vesicles, bullae, or a combination on the distal extremities (often including palms and soles) and face [msdmanuals.com]

    May be asymptomatic or cause pruritus and painful burning Distribution Symmetrical distribution Affects backs of hands and feet first → spreads proximally and can affect the entire body, including palms and soles Mucus membrane involvement EM minor: no [amboss.com]

Gastrointestinal

  • Vomiting

    Symptoms include fever, malaise, sore throat, cough, vomiting, diarrhea, arthalgia, and myalgia. [icd9data.com]

    Systemic side effects of PCV13 include chills, fever, headache, vomiting, fatigue, arthralgia, myalgias, decreased appetite, and diarrhea, whereas its cutaneous adverse reactions are local injection site reactions, Sweet's syndrome, and deep morphea. [ncbi.nlm.nih.gov]

    Symptoms include fever, malaise, sore throat, cough, vomiting, diarrhea, arthralgia, and myalgia. [fpnotebook.com]

    Adverse reactions The most commonly reported adverse reactions were cough and vomiting. [revcovi.com]

  • Nausea

    She reported discharge from the vaginal and ocular lesions, as well as subjective fevers, blurry vision, dysuria, nausea, and vomiting. She denied cough. [consultant360.com]

    Diarrhea, abdominal pain and nausea are the most common adverse effects associated with sorafenib [2,5] and usually become more tolerable with time of use. [elsevier.es]

    Their main adverse effects are gastrointestinal symptoms as diarrhea, nausea, abdominal cramps and flatulence, which are usually light or moderate and transient. [scielo.br]

Jaw & Teeth

  • Aphthous Stomatitis

    Oral lesions must be distinguished from aphthous stomatitis, pemphigus, herpetic stomatitis, and hand-foot-and-mouth disease. [merckmanuals.com]

    • In the presence of oral lesions, – Aphthous stomatitis, – Contact dermatitis or stomatitis – Acute necrotizing gingivitis – Pemphigus, – Dermatitis herpetiformis, – Bullous lichen planus, – herpes zoster, – chickenpox – Toxic epidermal necrolysis [slideshare.net]

  • Sore Mouth

    A very sore mouth may lead to dehydration and poor nutrition. Genitourinary lesions may result in urinary retention. If the eye is involved it is important to prevent infection or conjunctival scarring. [patient.info]

Musculoskeletal

  • Arthralgia

    Systemic side effects of PCV13 include chills, fever, headache, vomiting, fatigue, arthralgia, myalgias, decreased appetite, and diarrhea, whereas its cutaneous adverse reactions are local injection site reactions, Sweet's syndrome, and deep morphea. [ncbi.nlm.nih.gov]

    Symptoms include fever, malaise, sore throat, cough, vomiting, diarrhea, arthralgia, and myalgia. [fpnotebook.com]

    The acute disease is often associated with fever, malaise, and arthralgias. [medical-dictionary.thefreedictionary.com]

    […] and can affect the entire body, including palms and soles Mucus membrane involvement EM minor: no/minimal involvement of mucus membranes EM major: painful ulcers of the oral (most common), ocular, genital mucosa Further symptoms: fever, myalgia, and arthralgia [amboss.com]

Eyes

  • Eye Pain

    […] burning, itching, and discharge Eye pain Mouth sores Vision problems There are two forms of EM: EM minor usually involves the skin and sometimes mouth sores. [nlm.nih.gov]

    They may or may not itch and may be accompanied by fatigue, fever, and joint pain. Some people also develop abnormalities of vision, including dry or bloodshot eyes, eye pain, burning or itching, or mouth sores. [drweil.com]

    Symptoms include fever, chills, fatigue, bloodshot eyes, eye pain, mouth sores, dry eyes, vision problems, general ill feeling, weakness and painful joints. Skin lesions will appear on the skin, a few to hundreds could erupt. [advanced-dermatology.com.au]

  • Conjunctival Injection

    In 6th day of fever the child showed mild bilateral bulbar conjunctival injection without exudate. Elevated antistreptolysin O antibody titer confirmed recent streptococcus pyogenes infection. [ijponline.biomedcentral.com]

    In 6 th day of fever the child showed mild bilateral bulbar conjunctival injection without exudate. Elevated antistreptolysin O antibody titer confirmed recent streptococcus pyogenes infection. [ncbi.nlm.nih.gov]

Skin

  • Eruptions

    Hebra disease - an acute eruption of macules, papules, or subdermal vesicles presenting a multiform appearance. [medical-dictionary.thefreedictionary.com]

    The eruption is often preceded by primary or reactivated Herpes Simplex Virus infection or that of Mycoplasma pneumoniae. [pathwaymedicine.org]

    In our patient, AB, an acquired relapsing eruption characterized by a papulovesicular dermatitis along BL, 7,8 would be the main differential diagnosis. [journals.lww.com]

    Causes include infections (most commonly herpes simplex virus), drugs (e.g., sulfonamides, anticonvulsants, and antibiotics), malignancies, and collagen vascular disorders A skin and mucous membrane disease characterized by an eruption of macules, papules [icd9data.com]

    Erythema multiforme–like eruption on the patient's arm. [5minuteconsult.com]

  • Blister

    M pneumoniae patients were older at presentation (P = .03) and more frequently had sore throat (P < .01) and atypical targets with central blistering (P < .01). [ncbi.nlm.nih.gov]

    blisters or sores on your lips and lining of your mouth Rash may go away on its own after several weeks but may keep coming back. [merckmanuals.com]

    EM starts abruptly over two to three days as dull, red spots, which later become raised or blistered. The condition progresses into classic target-like lesions consisting of concentric rings. [dermcoll.edu.au]

  • Skin Lesion

    We report a ten year-old healthy male child who developed skin lesions of both palms and soles associated with oral ulcerative lesions. [ncbi.nlm.nih.gov]

    Diagnostic Criteria for Erythema Multiforme minor Clinical Course Acute, self-limited or episodic outbreak Duration Attack between days to weeks in evolution Primary skin lesions Symmetrically appearing discrete, round erythematous skin lesions lasting [cancertherapyadvisor.com]

    Fungal urinary tract infection could have been related to the development of the skin lesions. [jcnonweb.com]

    All 3 patients were immunocompetent, and the inflammatory infiltrate in the skin lesions lacked plasma cells. [medicaljournals.se]

    There may be multiple skin lesions with rashes that show color or texture change. [dovemed.com]

  • Urticaria

    Tags: burn Erythema Multiforme Rash Stevens Johnson TEN urticaria urticarial multiforme viral illness Sean M. Fox I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. [pedemmorsels.com]

    Early in the course, these lesions may easily be mistaken for urticaria. As the lesions enlarge, they form round or irregularly shaped plaques. [medicinenet.com]

    L50.6 Contact urticaria L50.8 Other urticaria L50.9 Urticaria, unspecified L51 Erythema multiforme L51.0 Nonbullous erythema multiforme L51.1 Stevens-Johnson syndrome L51.2 Toxic epidermal necrolysis [Lyell] L51.3 Stevens-Johnson syndrome-toxic epidermal [icd10data.com]

    EM is very commonly confused with annular urticaria (hives). Urticaria is far more common than EM. [cancertherapyadvisor.com]

  • Skin Rash

    Within all the people who go to their doctor with erythema multiforme, 92% report having skin rash, 67% report having fever, and 44% report having itching of skin. [symcat.com]

    It consists of a skin rash that involve no more than one mucosal surface. The sudden onset will progress rapidly as symmetrical lesions with circular color changes in some or all of the lesions. Rash will spread towards center or trunk of the body. [en.wikipedia.org]

    Erythema multiforme (EM) refers to a form of skin rash. The term 'multiforme' refers to the fact that its appearance can vary on the person and may vary from person to person. [healthcentral.com]

    It occurs with any skin injury, infection or inflammation. erythema ab igne that due to exposure to radiant heat. erythema chronicum migrans the early skin rash at the site of the tick bite which infects humans with Borellia burgdorferi (Lyme disease) [medical-dictionary.thefreedictionary.com]

    Blister-like skin rash with intense itching. Mouth ulcers (mucosal involvement suggests Stevens-Johnson syndrome). Examination Target-shaped lesions, usually over the limbs. [syrianclinic.com]

Neurologic

  • Hyperactivity

    Central nervous system: development may be slower than in healthy children and there may be behavioural and psychological problems, such as hyperactivity and poor social behaviour. Hearing: deafness is sometimes a problem. [gosh.nhs.uk]

Workup

Laboratory tests are usually not required to confirm the presence of EM as a diagnosis is made clinically [8]. Imaging studies are not needed in most cases either, although chest radiography can be useful if a patient has respiratory symptoms or an underlying pulmonary infection.

If the condition is considered to be severe, there may be specific tests conducted. Elevation of the erythrocyte sedimentation rate and leukocytosis may be present. Cultures including blood cultures, mucosal lesions cultures, and sputum cultures are also taken in these cases.

Treatment

Before any treatment for EM can be done, the underlying cause of the disorder will need to be identified. If erythema multiforme is drug-induced, the offending medication must be withdrawn. If the cause is an infection, that condition should be treated first.

Symptomatic treatment includes oral antihistamines, analgesics, and mouthwashes [9]. Various topical steroids can be used. If the eyes are affected, topical lubricants and supportive care can be used in treatment. Recurrent and severe forms require further treatment, frequently with continuous oral acyclovir.

Prognosis

In most cases EM is considered to be self-limited. The lesions that are associated with the minor form of EM usually resolve over two to three weeks without scarring [6]. It is common for EM minor to recur especially in patients with a condition caused by the herpes simplex virus.

The more severe form, EM major, is associated with a mortality rate of under five percent. Mortality in relation to EM major is usually due to sepsis or penumonia. The associated lesions usually heal without scarring but they can cause postinflammatory hypopigmentation or hyperpigmentation and rarely scarring due to a secondary infection. Ocular involvement may lead to keratitis, conjunctival scarring, and visual impairment in rare cases.

Etiology

The disorder is believed to be caused by a number of different factors that can vary from case to case [3]. In about half of the reported cases of the disorder there is no one identifiable factor behind the occurrence of the EM and it is considered to be idiopathic. Certain medications such as antibiotics, anticonvulsants or antituberculoids can trigger the the disorder and bacterial, viral, parasitic and fungal infections are also a common cause of the condition. There are records of EM related to post-vaccinations including the Bacille Calmette-Guerin vaccination, the oral polio vaccine, and the tetanus/diphtheria vaccine. Erythema multiforme may also be associated with radiation, autoimmune diseases, and malignancies. 

Epidemiology

On an international scale EM is uncommon and the incidence is reported to be 00.1% to 1% of the population. Erythema multiforme occurs predominantly in adults between 20 and 40 years of age, although it can affect individuals of all ages. Men and women are equally affected.

Pathophysiology

It is believed that EM is an immunological disorder that results from a hypersensitive reaction triggered by various etiologic agents. However, the complete pathogenesis of erythema multiforme is not yet fully understood [5].

The pathogenesis of erythema multiforme due to herpes simplex virus has been investigated and is consistent with a delayed-type hypersensitivity reaction. It is however unclear, whether a similar mechanism is responsible for the development of the disorder related to other etiologic factors. 

There may be a genetic predisposition in the development of EM. Especially HLA-DQB1*0301 was detected more frequently in patients with erythema multiforme. Other HLA types may play a role as well.

Prevention

There is no way to prevent EM from occurring [10]. When a person develops EM, it is important to figure out what triggered the disorder. This will allow them to better prevent recurrences.

Summary

Erythema multiforme (EM) is a self-limited, acute skin condition possibly mediated by of a type IV hypersensitivity that is associated with various infections or drug exposure [1]. This disorder can vary in severity based on the affected person and can be triggered by different catalysts. EM is divided in two forms: Erythema multiforme minor and erythema multiforme major [2].

Patient Information

Erythema multiforme (EM) is a skin condition that is caused by various factors, but is mainly caused by a reaction to a medication, vaccine or infection. There are two forms of the disorder, a minor and a major form. Each form varies in intensity and can take anywhere from one week to six weeks to clear up. The minor form of EM is a slight skin disorder that clears up quickly while the major form of EM can be severe and, in some cases, life threatening. EM is characterized by lesions that look like targets. They appear on the extremities and inside of the mouth. The severity of the individual case will usually determine the type of medical treatment needed.

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