Erythema multiforme is a rare skin condition characterized by targetoid lesions.
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]
- Malaise
Symptoms include fever, malaise, sore throat, cough, vomiting, diarrhea, arthalgia, and myalgia. [icd9data.com]
[…] mycoplasma, typhoid), drugs (penicillin, phenylbutazone, phenytoin, salicylates, sulfa), carcinoma / lymphoma, or collagen vascular disorders Affects skin (distal extremities, palms, soles) and mucous membranes with target lesions Also sore throat and malaise [pathologyoutlines.com]
The case of a 36 year old male patient presenting to emergency of HNAL with fever, malaise, itching and painful ulcer type lesions in oral cavity, multiple target lesions in upper and lower limbs, penis, and perianal region is reported. [scielo.org.pe]
Symptoms include fever, malaise, sore throat, cough, vomiting, diarrhea, arthralgia, and myalgia. [fpnotebook.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]
- Hodgkin Lymphoma
Physical factors: radiotherapy, cold, sunlight Others: collagen diseases, vasculitides, non-Hodgkin lymphoma, leukaemia, multiple myeloma, myeloid metaplasia, polycythemia EM minor is regarded as being triggered by HSV in almost all cases.[5] A herpetic [en.wikipedia.org]
lymphoma, leukemia, multiple myeloma, myeloid metaplasia, and polycythemia Physical or mechanical factors, such as tattooing, radiotherapy, cold, and sunlight Foods, including salmon berries and margarine Malignancy Hormonal [emedicine.com]
- Sepsis
A full sepsis work-up was performed in each infant. The fever settled within 1 day of admission in all of the infants, and was attributed to postvaccination fever, with negative microbiological investigations. [pediatr-neonatol.com]
The most common cause of death in patients with Stevens-Johnson syndrome is sepsis. [lecturio.com]
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. [symptoma.com]
The complications of Erythema Multiforme could include: Skin infection Sepsis: It is a body-wide infection due to blood poisoning Shock, due to the loss of body fluids The skin lesions can occasionally cause: Myocarditis (heart inflammation) Pneumonitis [dovemed.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]
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. [msdmanuals.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]
- 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]
- Nikolsky's Sign
[…] can aid diagnosis Differential Diagnosis SJS/TEN (flat lesions, tender, always with mucous involvement, + Nikolsky sign) Urticaria (annular lesions) Erythema nodosum Viral exanthems Treatment Supportive care Corticosteroids if severe Treat underlying [medbullets.com]
’s sign Febrile Staphylococcal scalded skin syndrome (children) Toxic epidermal necrolysis/SJS (adults) Afebrile Toxic epidermal necrolysis Negative Nikolsky’s sign Febrile Toxic shock syndrome Kawasaki disease Scarlet fever DRESS syndrome Cellulitis [wikem.org]
Nikolsky sign is negative. Acute and convalescent mycoplasma titers may be employed to help to establish this diagnosis in the correct clinical scenario. [visualdx.com]
EM major (involvement of mucous membranes and systemic signs) Corticosteroids Ophthalmology consult if ocular involvement EM minor (no mucous membrane involvement and no systemic signs) is usually self-limited Supportive care Early treatment with acyclovir [smartypance.com]
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.
HLA Type
- HLA-A1
[…] symptoms; Transformation into SLE with moderate disease activity in 10-15%; ACR criteria formally met in 50%; ANA in 60-80%; Anti-Ro/SSA antibodies 70-90%; anti-la/SSB antibodies in 30-50%; Positive rheumatoid factor (>30%); Immunogenetic disposition: HLA-A1 [oatext.com]
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.