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Rashmi Agarwal, Amit Mhapuskar, Manjula Hebbale, Meenal Tepan, Ayushee

The document reports a case study of a 30-year-old male patient with erythema multiforme minor. The patient presented with multiple oral ulcers and lesions that were managed with topical corticosteroids, resulting in complete remission. The document also provides background information on erythema multiforme, including causes, pathogenesis, and management.

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0% found this document useful (0 votes)
58 views5 pages

Rashmi Agarwal, Amit Mhapuskar, Manjula Hebbale, Meenal Tepan, Ayushee

The document reports a case study of a 30-year-old male patient with erythema multiforme minor. The patient presented with multiple oral ulcers and lesions that were managed with topical corticosteroids, resulting in complete remission. The document also provides background information on erythema multiforme, including causes, pathogenesis, and management.

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Journal of International Dental and Medical Research ISSN 1309-100X

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Erythema Multiforme Minor


Rashmi Agarwal, and et al

Erythema Multiforme Minor - Report of a Case with Review of Literature


Rashmi Agarwal1*, Amit Mhapuskar2, Manjula Hebbale3, Meenal Tepan4, Ayushee1
1. Post graduate student, Department Of Oral Medicine And Radiology, Bharati Vidyapeeth Deemed University Dental College And Hospital,
Pune.
2. M.D.S, Professor and H.O.D, Department Of Oral Medicine And Radiology, Bharati Vidyapeeth Deemed University Dental College And
Hospital, Pune.
3. M.D.S, Reader, Department Of Oral Medicine And Radiology, Bharati Vidyapeeth Deemed University Dental College And Hospital, Pune.
4. M.D.S, Asst. Professor, Department Of Oral Medicine And Radiology, Bharati Vidyapeeth Deemed University Dental College And
Hospital, Pune.

Abstract
Erythema Multiforme (EM) is an acute, immunemediated condition characterized by the
appearance of distinctive target like lesions on the skin. It is triggered by a variety of conditions
including infections, drug use, vaccines etc. It has a spectrum of manifestations from mild to
fulminating variants creating a diagnostic dilemma. The incidence of EM has been estimated to be
between 0.01 and 1%.Prevalence of oral EM varies from 35% to 65% among patients with skin
lesions. However, in patients where EM was diagnosed by oral lesions, prevalence of skin lesions
ranged only from 25% to 33%.
We report a case of erythema multiforme minor in a 30 year old male patient managed with
topical corticosteroids with complete remission.
Case report and Review (J Int Dent Med Res 2016; 9: (2), pp. 129-132)
Keywords: erythema multiforme, oral ulcers, acute onset, topical corticosteroids.
Received date: 02 March 2016
Accept date: 11 March 2016
Introduction
Erythema multiforme is an acute
mucocutaneous hypersensitivity reaction with a
variety of etiologies. Ferdinand Von Hebra
described erythema multiforme (EM) in the year
1866 as a self-limited and acute skin disease that
is symmetrically scattered on the extremities with
a typical recurring concentric pattern in the form
of target lesion.1
It is characterized by a skin eruption, with
or without oral or other mucous membrane
lesions. It can be induced by drug intake or
several infections, in particular herpes simplex
virus (HSV) infection, which has been identified
in up to 70% of erythema multiforme cases.2,3 It
comprises of variants in a range from a mild,
exanthematous, self-limited and cutaneous
*Corresponding author:
Dr. Rashmi Agarwal
Post graduate student,
Department Of Oral Medicine And Radiology,
Bharati Vidyapeeth Deemed University
Dental College And Hospital, Pune.
E-mail: [email protected]

Volume 9 Number 2 2016

variant with least oral involvement known as EM


minor; to a more severe, fulminating and
progressive
variant
with
an
extensive
mucocutaneous epithelial necrosis known as
Stevens-Johnson syndrome (SJS) and toxic
epidermal necrolysis (TEN); hence the name
multiforme.4 The clinical classification of these
disorders is variable, thus making definitive
diagnosis difficult.
Early
recognition
and
prompt
management will benefit the patients. We present
a classic case of extensive erythema multiforme
minor with a follow up of complete remission.
Case Report
A 30 year old male patient visited the
department with a chief complaint of multiple
ulcers on oral mucosa with pain in the mouth
since one week. The pain was sudden in onset,
continuous in nature and pricking type which
aggravated on eating or drinking. The lesions first
appeared on the palate and after a few days
similar lesions came everywhere in the mouth.
Patient also noticed blood in his sputum. As said
by the patient the lesions were present only on
the oral mucosa; no other mucosa or skin was
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involved. He also gives a history of mild fever for


3 days before the appearance of the lesions. No
history of previous episodes of such lesions was
present. No relevant medical, dental, stress or
drug history was known. Patient gives a history of
alcohol consumption three to four times a week.
No other tissue abuse habit was reported.
Extraoral examination showed presence
of multiple erosions with bloody crustings
involving the entire lower lip. Introral examination
revealed multiple large confluent erythematous
ulcerations with irregular margins and covered
with yellowish pseudomembrane on the entire
surface of right and left buccal mucosa, upper
and lower labial mucosa, vestibular mucosa, floor
of the mouth, palate, lateral surface, tip and
ventral surface of the tongue (fig. 1).

Erythema Multiforme Minor


Rashmi Agarwal, and et al

stained section showed that the epithelium was


ulcerated with presence of subepithelial vesicle
and necrotic basal keratinocytes. The connective
tissue showed mature connective tissue stroma
with chronic inflammatory infiltrate. Perivascular
inflammatory cell infiltrate was also evident (fig.
2).

Figure

2. H & E section (40x) shows


subepithelial vesicle and necrotic basal
keratinocytes.

Figure 1. Bloody crustings involving the entire


lower lip and multiple large confluent
erythematous ulcerations with irregular margins
and covered with yellowish pseudomembrane on
the entire oral mucosa.
On palpation the ulcers were shallow,
tender, soft in consistency and bled on
provocation. There was no presence of intraoral
bullae on the mucosa. The history of acute onset
of the lesions with mild fever and presence of
bloody crustings on the lip led to a classic
provisional diagnosis of erythema multiforme
minor. The differential diagnosis considered was
of pemphigus vulgaris, erosive lichen planus and
allergic stomatitis.
Incisional biopsy was performed from the
right buccal mucosa of the lesional and the
perilesional site. The tissue was stained with
hematoxylin
and
eosin
and
direct
immunofluorescence was done. The H & E
Volume 9 Number 2 2016

Figure 3. recall visit after 7 days shows healing


of the lesions.
The overall picture was suggestive of
erythema multiforme. Direct immunofluorescence
revealed non- specific deposition of IgG in the
epithelium. IgA, IgM and C3 were negative.
Correlating history, clinical findings and
histopathology a final diagnosis of erythema
multiforme was made. The patient was
prescribed with triamcinolone acetonide 0.1% to
be applied on the entire oral mucosa three times
a day after meals for 15days. The patient was
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recalled after 7 days (fig. 3) and then after 15


days (fig. 4). The patient responded well to the
therapy given with 90% of the lesions healed
after 7 days and complete healing was seen after
15days.

Figure 4. Recall visit after 15 days shows


complete remission of the lesions.
Discussion
The term erythema multiforme (EM) is a
clinical condition which reflects the broad
morphological spectrum of the lesions.5 The peak
age at presentation is between 20 and 40 years
although 20% of cases occur in children.6 The
oral lesions are accompanied by rapidly rupturing
vesicles and bullae leading to diffuse sloughing
and ulceration of the whole surface of the skin
and mucous membrane.7
Erythema multiforme has been reported
to be triggered by numerous agents, particularly
viruses, especially herpes simplex virus (HSV)
but other herpesviruses (varicella-zoster virus,
cytomegalovirus,
Epstein-Barrvirus),
adenoviruses, enteroviruses (Coxsackie virus B5,
echoviruses), hepatitis viruses (A, B and C),
influenza,
paravaccinia,
parvovirus
B19,
poliomyelitis, vaccinia and variola have all been
implicated.8 Drugs such as sulphonamides (e.g.
co-trimoxazole), cephalosporins, aminopenicillins,
quinolones, chlormezanone, barbiturates, oxicam
non-steroidal
anti-inflammatory
drugs,
anticonvulsants, protease inhibitors, allopurinol or
even corticosteroids may be implicated. Food
additives or chemicals such as benzoates,
nitrobenzene, perfumes have also been reported
as aetiological agents.9 Over 50% of patients
have unknown aetiology with stress or emotional
factor as the second largest category.7 In the
Volume 9 Number 2 2016

Erythema Multiforme Minor


Rashmi Agarwal, and et al

present case the patient gave a history of mild


fever before the onset of the intraoral lesions
which could suggest a viral etiology.
The exact pathogenesis is unknown. It
has been suggested that EM results from T-cellmediated immune reaction to the precipitating
agent, which lead to a cytotoxic immunological
attack on keratinocytes that express non selfantigens,
which
subsequently
leads
to
subepithelial and intra-epithelial vesiculation; that
causes widespread blistering and erosions. A
better understanding of the molecular and
immunologic events underlying HSV-associated
EM (HAEM) and their main differences with
respect to drug induced EM has been provided
by recent studies. It is suggested that disease
development begins with HSV infection of
epithelial skin cells, and subsequently circulating
mononuclear CD34 cells (Langerhans cell
precursors). This transports the HSV-DNA
fragments to distant skin sites, where an immune
mediated epidermal damage occurs due to
production of interferon- (IFN-). Conversely, in
drug-induced EM, tumour necrosis factor alpha
(TNF-) induces keratinocyte apoptosis which is
released from keratinocytes, macrophages, and
monocytes causing the tissue damage. A subset
of EM patients has been reported to have
autoantibodies against desmoplakins I and II and
antiepidermal autoantibodies. In addition to a
cellular immune response, humoral immune
mechanisms may be involved in the
pathogenesis of EM-like disease.10
Conclusions
The management of EM can be difficult.
An important element in EM treatment is the
discontinuation of all inciting factors. In addition,
disease management depends on other factors,
such as the presence of mucosal disease, the
development of recurrent disease and overall
disease severity. Mild forms usually heal in 26
weeks; local wound care, topical analgesics or
anesthetics for pain control and a liquid diet are
often indicated in these situations. For more
severe cases, intensive management with
intravenous fluid therapy may be necessary. Oral
antihistamines and topical steroids may also be
necessary to provide symptom relief. Systemic
corticosteroids have been used successfully in
some patients.11
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Erythema Multiforme Minor


Rashmi Agarwal, and et al

Declaration of Interest
The authors report no conflict of interest
and the article is not funded or supported by any
research grant.
References
1. Shafer WG, Hine MK, Levy BM. Text Book of Oral Pathology.
6th ed. New Delhi: Elsevier; 2009.
2. Al-Johani KA, Fedele S, Porter SR. Erythema multiforme and
related disorders. Oral Surg Oral Med Oral Pathol Oral Radiol
Endod 2007;103:642-54.
3. Farthing P, Bagan JV, Scully C. Mucosal diseases series:
Number IV: Erythema multiforme. Oral Dis 2005;11:261-7.
4. Neville BW, Damm DD, Allen CM. Oral and Maxillofacial
Pathology. 3rd ed. St. Louis, Missouri: Saunders Elsevier; 2009
5. Aurelian L, Ono F, Burnett J. Herpes simplex virus (HSV)associated erythema multiforme (HAEM): a viral disease with
an autoimmune component. Dermatol Online J. 2003
Feb;9(1):1.
6. Carrozzo M, Togliatto M, Gandolfo S. Erythema multiforme. A
heterogeneous pathologic phenotype. Minerva Stomatol. 1999
May;48(5):217-26.
7. BV Shobha, SP Mosby, R Thanuja. Erythema Multiforme - A
Case Report. JIDA; 2010:4(12).
8. Tatnall FM, Schofield JK, Leigh IM. A double-blind, placebocontrolled trial of continuous acyclovir therapy in recurrent
erythema multiforme. Br J Dermatol. 1995 Feb;132(2):267-70.
9. Porter SR, Scully C. Adverse drug reactions in the mouth. Clin
Dermatol. 2000 Sep-Oct;18(5):525-32.
10. Firoozeh Samim, Ajit Auluck, Christopher Zed, P. Michele
Williams. Erythema Multiforme A Review of Epidemiology,
Pathogenesis, Clinical Features, and Treatment. Dent Clin N
Am 57 2013; 583596
11. Kamala KA, Ashok L, Annigeri RG. Herpes associated
erythema multiforme. Contemp Clin Dent 2011;2:372-5.

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