Erythema Multiforme

ERYTHEMA
MULTIFORME
CONTENTS
1-INTRODUCTION
2-ETIOLOGY AND PATHOGENESIS
3-CLINICAL FINDINGS
4-ORAL MANIFESTATIONS
5-DIFFERENTIAL DIAGNOSIS
6-LABORATORY FINDINGS
7-MANAGEMENT
INTRODUCTION
Erythema multiforme (EM) is an acute, self-limited, inflammatory
mucocutaneous disease that manifests on the skin and often oral mucosa,
although other mucosal surfaces, such as the genitalia, may also be involved.
It represents a hypersensitivity reaction to infectious agents (majority of cases)
or medications.
It is classified as -
EM minor if there is less than 10% of skin involvement and there is minimal to
no mucous membrane involvement.
EM major has more extensive skin involvement, with the oral mucosa and other
mucous membranes.
However, there is likely a subset of EM that affects the oral mucosa only without
skin involvement. Fulminant forms of erythema multiforme labeled Stevens-
Johnson syndrome (SJS) and toxic epidermal necrolysis. However, more recent
studies suggest that EM is etiopathogenetically distinct from those two latter
conditions .
ETIOLOGY AND PATHOGENESIS
• EM is a hypersensitivity reaction, and the most common inciting factor is infection,

particularly with HSV.
• Drug reactions to NSAIDS, anticonvulsants, or other drugs play a smaller role.
• Cases of oral EM precipitated by benzoic acid, a food preservative, have been
reported.
• A condition reported as mycoplasma-induced rash and mucositis (MIRM), appears
similar to EM and also stevens jhonson syndrome in that patients, present with
extensive oral and mucosal lesions but usually less prominent skin lesions.
• However, infection with Chlamydia, pneumonia may also cause similar lesions.
CLINICAL FEATURES
EM generally affects those between ages 20 and 40 years, with 20% occurring in
children.
Patients with recurrent EM have an average of 6 episodes a year (range 2-24), with
a mean duration of 9.5 years; remission occurred in 20% of cases.
There may be a presence of fever, malaise, headache, sore throat and cough. These
symptoms suggest a viral (especially respiratory tract) infection.
Skin lesions appear rapidly over a few days and begin as red macules that become
papular, starting mainly in the hands and moving towards the trunk in a
symmetrical distribution.
The skin lesions may take several forms-hence the term multiforme is given.
The classic skin lesion consists of a central blister or necrosis with concentric rings
of variable color around it called "target" or "iris" lesion of EM.
Image 1. presence of central blister surrounded by an edematous ring Image 2. drug induced erythema multiforme
which is further surrounded by erythematous border.
ORAL MANIFESTATIONS
• The oral findings in EM range from mild erythema and erosion to large painful
ulcerations. When severe, ulcers may be large causing difficulty in eating, drinking,
and swallowing, and patients with severe EM may drool blood-tinged saliva.
Extensive lip involvement with inflammation, ulceration, and crusting is common.
• Oral lesions are present in 23 to 70% of patients with recurrent EM. The most
commonly affected sites are the lips (36%), buccal mucosa (31%), tongue (22%),
and labial mucosa (19%). Genital and ocular sites are affected in 17% of cases.
• The concept of pure oral EM is not universally accepted since some dermatologists
believe that the characteristic appearance and distribution of skin lesions are the
sine qua non for the diagnosis of EM. Intraoral lesions are irregular bullae, erosions,
or ulcers surrounded by extensive erythema. Crusting and bleeding of the lips is
present
Image 3 Image 4- drug induced em
• Recurrent erythema multiforme
DIFFERENTIAL DIAGNOSIS
• HSV gingivostomatitis with its viral features and erosions and ulcerations may
resemble oral EM, but these lesions culture positive for HSV and do not usually
present with the typical skin rash. Oral ulcers of HSV are usually smaller, well
circumscribed, and clustered, whereas EM lesions are larger and irregular.
Autoimmune vesiculobullous disease such as pemphigus and pemphigoid may
have oral ulcers and skin lesions, although skin lesions are bullous in nature and
not maculopapular.
• Hemorrhagic crusts on the lips are seen in paraneoplastic pemphigus (associated
with malignancies) and Stevens Johnson syndrome. The latter may be difficult to
distinguish from EM.
LABORATORY FINDINGS
• The diagnosis is made mainly on clinical findings and a recent history of HSV
infection.
• IgG and IgM levels not a reliable test although they may be suggestive. A negative
IgG level rules out HSV as an etiologic agent.
• Early lesions show lymphocytes and histiocytes in the superficial dermis around
superficial dermal vessels.
MANAGEMENT
• Mild oral EM can be managed with systemic or topical analgesics for pain and supportive care
since the disease is self- limiting and resolves within a few weeks.
• More severe cases are usually managed with systemic corticosteroids although patients often
worsen on discontinuation of steroid therapy.. Topical steroids may also help solve lesions. If
suspected of being HSV-associated should be treated with anti- viral medications, and
prophylaxis may prevent recurrences.. Treatment with acyclovir at the first sign of disease in
recurrent EM controls disease in approximately half of patients. Other treatment modalities
include dapsone, hydroxychloroquine, azathioprine, methotrexate, and intravenous
immunoglobulin.
• Continuous acyclovir at 400 mg twice a day prevents development of EM in most patients with
hSV-related EM, whereas EM not related to HSV responded well to azathioprine (100-150
mg/day).
REFERENCES-
1- BURKET’S ORAL MEDICINE

2- www.sciencedirect.com
3- www.researchgate.net
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ERYTHEMA

• EM is a hypersensitivity reaction, and the most common inciting factor is infection,

1- BURKET’S ORAL MEDICINE

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