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Sathawane et. al : Erythema Multiforme Major : Case Report and Review
ERYTHEMA MULTIFORME MAJOR: CASE REPORT AND
REVIEW OF LITERATURE
Dr. R. S. SATHAWANE1,
Dr. SAMIKSHA TRIPATHI2, Dr. ABHIJEET DEOGHARE3
1.
PROFESSOR, HEAD OF DEPARTMENT, Department of Oral Medicine and Radiology
2.
POST GRADUATE STUDENT, Department of Oral Medicine and Radiology
3.
READER, Department of Oral Medicine and Radiology
CHHATTISGARH DENTAL COLLEGE AND RESEARCH INSTITUTE, RAJNANDGAON (C.G.), INDIA.
ADDRESS FOR CORRESPONDENCE
Dr. Samiksha Tripathi, D/o Chandra Kant Tripathi, Manager, Ultratech Cement, QTR No.- 3064, Grasim
Vihar, Rawan, Dist - Baloda Bazar, Chhattisgarh. Pincode – 493196.
Email id – [email protected]
Phone no. – +919009904195, +919826725406
ABSTRACT
Erythema multiforme is an acute mucocutaneous disorder that occurs with varying degrees of blistering and
ulceration. We report a case of major erythema multiforme managed with systemic steroids. A 45-year-old male had
cutaneous target lesions and ulcerative lesions throughout the oral cavity and lips, which had been diagnosed as
erythema multiforme major. This episode was related to neither drug intake nor herpetic infection, which suggests
that the erythema multiforme was of idiopathic origin. This hypothesis was supported by negative serology for
herpes simplex virus. Excisional biopsy of an intact bulla was performed and the diagnosis was confirmed as
erythema multiforme major. The patient was treated with Prednisolone in a tapering dose for 2 months to control and
completely cure the disease.
Key-words: Erythema multiforme, Immune disorder, Target lesion.
INTRODUCTION
Erythema multiforme (EM) is a rare, acute
immune-mediated mucocutaneous disorder, a
widespread hypersensitivity reaction, caused by
the appearance of cytotoxic T lymphocytes in the
epithelium that induce apoptosis in keratinocytes,
leading to satellite cell necrosis.1 Despite being
frequently caused by, or at least associated with,
infection or drug therapy, the pathogenic
mechanism of EM stays vague, and as a result
there are no evidence-based, reliably effective
therapies. Oral manifestations may occur
independently or precede cutaneous involvement.2
The present article discusses a case of 45-year old
male who was clinically and histopathologically
diagnosed as erythema multiforme and reviews
aspects of EM as relevance to dental practice.
CASE HISTORY
A 45-year old male patient presented to the
outpatient department of our institute with
complains of painful oral ulceration and
hemorrhagic crusts on the lips. History revealed
that complaints started around 2 months back.
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Sathawane et. al : Erythema Multiforme Major : Case Report and Review
Initially there was erythema in the oral cavity and
over lips, due to which patient experienced
burning and pain during mastication. Pain was
gradual in onset, moderate to severe in intensity,
continuous in duration, aching type. There was
no history of referred/radiating pain. Soon
vesicles and ulcers appeared at these sites.
Vesicles first appeared on lips, buccal mucosa
bilaterally, followed by hard palate and then onto
tongue and upper and lower labial mucosa.
Vesicles ruptured to form encrustations over lips.
History of itching and burning sensation present
in the perioral region. Also history of dysphonia,
odynophagia and dysarthria was present. No
history of any concomitant symptoms associated
with pain nor febrile episode was present. There
was no history of any drug intake before the onset
of these lesions. History of similar lesions starting
primarily in the hands, legs & then moving
centripetally toward the trunk, neck, inguinal,
genital area and on external nares and near inner
canthus of right eye 1 ½ months back. No history
of previous episodes of ulcerations elsewhere in
the body. Patient went to a private medical
practitioner, got medicated and was referred to the
college for further needful treatment.
On clinical examination, dark brown and red
colored encrustations were present on lips. Lips
were edematous and erythema was present around
encrustations (Fig. 1). Bleeding ulcers and
localized areas of erythema were present on hard
palate, buccal mucosa, tongue and gingivae (Fig.
2). None of the lymph nodes were palpable.
Diagnostically significant finding was the
presence of multiple target lesions on the trunk
(Fig. 3). Nikolsky's sign was negative. Other
systemic examination was normal. Clinically,
diagnosis of erythema multiforme was made.
Routine hematological investigations were within
normal range.
Biopsy from the lesion on
histopathological examination (H&E and PAS
stain) revealed intercellular edema, sub-basilar
separation of epithelium from underlying
connective tissue along with degeneration of basal
cells at few places and connective tissue showing
numerous bundles of collagen fibres, fibroblast,
muscle tissue and blood vessels surrounded by
dense chronic inflammatory cells (Fig. 4).
Oral prednisolone at the dose of 20 mg twice daily
was started along with local application of potent
steroid - Clobetasol ointment thrice daily. Also
oral supplementations were prescribed, to improve
the overall condition of the patient. Within 15
days, there was decrease in the severity of the
mucosal and skin lesions and prednisolone was
tapered over next 15 days and stopped after further
tapering till all lesions healed. Mouthwashes
consisting of local anesthetics and antiseptics
were added for symptomatic treatment.
Complete hematological investigations were
routinely done during the course of treatment
which were within normal limits.
In addition, no oral or skin lesions developed
during the 2 months of treatment, the patient is
still under follow-up and the disease is currently
under control (Fig. 5).
DISCUSSION
Erythema multiforme (EM) is an acute,
usually
self-limiting,
immune-mediated,
blistering, ulcerative condition affecting the skin
and/or mucous membranes, including the oral
cavity.2,3,4 EM has been classified into a
number of variants, mainly minor and major forms
(Table 1).2,3.
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Sathawane et. al : Erythema Multiforme Major : Case Report and Review
Type of
Erythema
multiforme
EM Minor
(EMm)
EM Major
(EMM)
Characteristic features
Skin lesions

Involves less than 10% of
the body surface area
Mucosal lesions

Uncommon

Most commonly oral
mucosa

Ocasionally EMm that
only affects the oral
mucosa may arise
Skin lesions

Involves less than 10% of
the body surface area but
more severe than EMm
Mucosal lesions

Involves two or more
mucous membranes with
more
variable
skin
involvement. Oral lesions
are usually widespread and
severe
`
EM arises as a result of immune-complex
mechanisms involving antigen-antibody reactions
that target small blood vessels in the skin or
mucosa. In approximately, 90% of cases, the
precipitating event relates to infection, with the
herpes simplex virus (HSV) playing a
Once in a while it may be associated with
prodromal symptoms, which typically occur 7 to
14 days before development of cutaneous lesion.
The classic skin lesion of EM is a target or iris
lesion or bull’s eye distributed symmetrically on
the extremities and trunk and characterized by
concentric erythematous rings separated by rings
of near normal color with lesion size ranging from
2 to 20 mm with central area of necrosis or
crusting. Less commonly macules, papules or
plaques are manifested. Complete recovery from
an EM attack typically occurs within 1 to 4
weeks, with transient hypo/hyperpigmentation.3,6
Oral mucosal lesions occur in more than 70% of
EM cases.3 The lesions show considerable
variability in the appearance, ranging from diffuse
oral erythema, to multifocal superficial
ulcerations, thus the term multiforme. Initially,
vesicles or bullae may be present, which rupture
causing hemorrhagic crustations. Any area of the
mouth may be involved, with buccal mucosa,
palate, and tongue being most frequently affected.
In most cases, lip lesions show hemorrhagic
crustations. There may be mild to severe oral and
perioral pain that may interfere with functional
activities like speech, eating, swallowing and fluid
intake, debilitating the health of the patient.
Intraoral and perioral lesions heal without
scarring.6
The diagnosis of EM is chiefly based on the
history
and
clinical
presentation,
as
histopathologic
features
and
laboratory
predominant role in 70% to 80% of cases.5 Other
triggering factors may include medications,
especially sulfonamides, NSAIDS, penicillins, and
investigations are nonspecific.2,3,4
anticonvulsants.3
lack of evidence2 EMm may respond to topical
corticosteroids. Patients with EMM ought to be
Corticosteroids are the most commonly used
drugs in the management of EM, regardless of
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Sathawane et. al : Erythema Multiforme Major : Case Report and Review
treated
with
systemic
corticosteroids
(prednisolone 0.5–1.0 mg/kg/day tapered over 7–
10 days) or azathioprine, or both or other
immunomodulatory
medications
such
as
cyclophosphamide, dapsone,
cyclosporine,
levamisole,
thalidomide
or
interferon-a.7
Cyclosporine given intermittently may control
recurrent EM.8
.
Fig. 1: Ulcers and hemorrhagic crusts on the
lower lip during the first episode of EM
Fig. 2: Bleeding ulcers and localized areas of
erythema on buccal mucosa, hard palate,
gingivae and tongue
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Sathawane et. al : Erythema Multiforme Major : Case Report and Review
Fig. 3: Characteristic target lesions seen
on the trunk, back, extremities
Fig. 4: Microphotograph (H&E, 10x) showing
histopathological features suggestive of EM
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Sathawane et. al : Erythema Multiforme Major : Case Report and Review
4. Osterne, Brito, Pacheco et al. Management of
Erythema Multiforme Associated with
Recurrent Herpes Infection: A Case Report.
www.cda-adc.ca/jcda • October 2009, Vol.
75, No. 8.
5.
Fig. 5: Intra-oral and Extra-oral post steroid
therapy photographs of the patient, showing
almost completely healed lesions
ACKNOWLEDGEMENT
We acknowledge the help and guidance from Dr.
Shivmurthy, Professor and Head, Department of
Oral and Maxillofacial Surgery and Dr. Gandhi,
Head of Department of General Surgery,
Chhattisgarh Dental College and Research
Institute, Rajnandgaon (C.G.).
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