rare case of multifocal epithelial hyperplasia of oral mucosa

http://dx.doi.org/10.5272/jimab.2013194.392
ISSN: 1312-773X (Online)
Journal of IMAB - Annual Proceeding (Scientific Papers) 2013, vol. 19, issue 4
RARE CASE OF MULTIFOCAL EPITHELIAL
HYPERPLASIA OF ORAL MUCOSA
Elitsa G. Deliverska1, Maria Dencheva2
1)Department of Oral and Maxillofacial surgery,
2)Department of Oral Imaging and oral diagnostic,
Faculty of dental medicine, Medical University, Sofia, Bulgaria
ABSTRACT:
Introduction: Multifocal papilloma virus epithelial
hyperplasia (MPVEH) is a rare disease of the oral mucosa
associated with the human papilloma viruses (HPV) 13 and
32. It occurs at any age, but children have the highest
incidence.
Purpose: To present a case of oral multifocal
papilloma virus epithelial hyperplasia with palatal tumour
and multiple metachronic primary cancers.
Material and methods: The medical history shows a
60 years female patient with complaints of asymptomatic
mass on the palatal mucosa, with a two years’ development.
The patient has been treated surgically for previous thyroid
cancer, endometrial cancer and cancer of the mammary
gland. An incisional biopsy has been performed in her left
buccal mucosa 10 years ago and histopathological
examination confirmed the diagnosis of MPVEH.
The palatal tumour was removed surgically by
electroexcision and the histological result was pleomorphic
adenoma of a small palatal salivary gland.
Conclusion: Papillomatosis is considered as a benign
neoplasm with an unpredictable course and with a low
probability of malignancy.We report of an unusual case of
MPVEH persistent for more than 30 years without
regression and with significant impact on a patient‘s quality
of life.
Key words: Multifocal papilloma virus epithelial
hyperplasia, palatal tumour, multiple primary tumours.
INTRODUCTION:
Multifocal epithelial hyperplasia (MEH) is also
described as verrucae of the oral cavity, focal epithelial
hyperplasia, Heck’s disease, and multifocal papilloma virus
epithelial hyperplasia. The condition was first noted in the
Spanish literature by March in 1881 and later by Stern [7]
and Helms.[9] In 1961, Heck came across the condition
again and the earliest English publications were by Archand
et al.[1] and Witkop and Niswander [15] in 1965. Heck
noted multiple papules spread throughout the oral mucosa
in an 11-year-old Navajo girl.[1, 4] A series of 19 pediatric
392
patients with intraoral papules was reported, subsequently
with the authors coining the term “focal epithelial
hyperplasia” to describe the intraoral papules found in
affected Indian children from the Unites States and Brazil
and an Eskimo child from Alaska.[1] In 1966, Hettwer and
Rodgers[8] noted focal epithelial hyperplasia in a Polynesian
girl and named the condition “Heck’s disease” with the
eponym enduring in the dental literature; however the name
of “multifocal epithelial hyperplasia” is the most
appropriate, as it describes the major features of the disease
(the presence of multiple lesions widespread on the oral
mucosa and the microscopically prominent hyperplasia of
the oral epithelium Multifocal epithelial hyperplasia (MEH)
is a rare disease of the oral mucosa usually associated with
the human papilloma viruses (HPV) 13 and 32.[11] It
presents as multiple nodular or papular lesions with a sessile
base, ranging in diameter from 0.1 cm to 1.0 cm, frequently
coalescing. Lesion colour varies from red to white,
depending on the presence and degree of frictional
keratinisation. The condition occurs at any age, but children
have the highest incidence. Multiple epithelial hyperplasia
appears mainly in childhood and adolescence between 3 and
18 years of age and tends to spontaneously regress, although
it can persist for many years. The condition is rare in people
living in urban society, with most cases noted in tribal
communities from remote Greenland and North, Central, and
South America.[9] It is more common among females.[14]
The lesions are usually painless, unless traumatized, and are
frequently found on the buccal mucosa, lower lip, and
commissures but also found on the mucosa of the upper lip,
tongue, hard palate, and gingival. The etiological factors
and pathogenesis of MEH are speculative, but mainly
associated with the human papilloma viruses (HPV) 13 and
32 type.
The histologic appearance of epithelial hyperplasia
varies with basal epithelial club-shaped projections that
occasionally anastomose horizontally (“bronze age battleaxe” appearance), koliocytes, and other intracellular changes
indicative of viral infection such as hyperchromatism and
enlargement of the nuclei. Human papillomavirus 13 and 32
DNA have been consistently detected in these lesions. The
http://www.journal-imab-bg.org
/ J of IMAB. 2013, vol. 19, issue 4/
degenerating epithelial cells resemble cells in various phases
of mitosis and are called mitosoid cells.[7] These are mostly
found in the upper part of the epithelium and in younger
lesions but are not consistently present, with dilated
capillaries and some lymphocytes evident in the connective
tissue.[12]
The differential diagnoses include condyloma
accuminatum, florid oral papillomatosis, Cowden’s
syndrome, Crohn’s disease, Darier disease, focal dermal
hypoplasia (Goltz syndrome), and white spongy naevus.[7]
CASE REPORT
A 60 years old female reffered to our department of
Oral and Maxillofacial surgery with a mass on palatal
mucosa with a two years development and with history of
multiple oral masses of papules lesions since 30 years. (Fig.
1) The examination revealing several asymptomatic,
exophytic, pink,sessile, smooth-surfaced nodules on the
dorsal surface of the tongue, lingual surface of the lower lip,
gingiva of upper and lower jaw and buccal mucosa. (Fig. 2,
Fig. 3).
Multiple papules and nodules merging into
“intestine”- like mucosa could be seen on the tongue,
retrocommissural area, inside upper and lower lip and,
gingival, buccal mucosa, bilaterally. The lesions were normal
mucosa coloured, raised, smooth and with crypts inside.
Medical history of the patient includes multiple
primary cancers.
The patient has been treated surgically for previous
- thyroid cancer,
- endometrial cancer and
-cancer of the mammary gland.
An incisional biopsy has been performed in her left
buccal mucosa 10 years ago and histopathological
examination confirmed the diagnosis of Multifocal Human
Papilloma Virus Epithelial Hyperplasia- MPVEN.
In our department of Maxillofacial surgery,
University Hospital ‘St. Anna’, Sofia the palatal tumour was
removed surgically by electroexcision and the histological
result was cystic adenoma of a small palatal salivary gland
(#3043/31.03.2013). The surgical border was 0.5 cm and
clinicaly and histologicaly the borders were clean.(Fig. 4)
The postoperative defect was covered by gauze, soaked with
Iodasept unguentum.(Fig. 5) Intraoperative and
postoperative period was without any complication. The
gauze was removed 7 days after surgery, and the
postoperative wound was healing secondary. The patient was
follow up.
DISCUSSION
Although MEH is predominantly found in certain
populations in America and Greenland,[7] it can be seen in
other geographic regions like sporadic cases. Host factors
such as immune suppression (HIV infection- unusual HPV
/ J of IMAB. 2013, vol. 19, issue 4/
types), genetic predisposition, malnutrition, and hygiene are
key putative etiological factors.[4, 6] It has been proposed
that a combination of these factors increases an individual’s
risk for MEH.[9] A genetic predisposition has been proposed
based on familial occurrences and ethnic predilections.[13]
There have been multiple reports of the disease occurring
in different ethnic groups living in poverty such as Indian
communities from North, Central, and South America as
well as in Inuit from Greenland.[15] A genetic predisposition
may render an individual’s immune system susceptible to
acquiring certain types of HPV infection.[5] The clinical
course of MEH is benign and associated with HPV serotypes
with minimal carcinogenic risk; therefore, aggressive therapy
is usually not indicated.[14, 10] Removal of MEH lesions
is indicated in patients who have significant functional
disturbance or aesthetic concern. Current therapeutic options
include surgical excision, cryosurgery, electrocoagulatory
(carbon dioxide) lasers, interferon-_ injections, topical
application of interferon-_ or podophyllin, topical and
systemic retinoids, vitamins,[3] and diode laser removal.[2]
Ideal treatment for symptomatic lesions in pediatric patients
is one that is noninvasive and has minimal or no systemic
side effects. The patient in this case has been informed of
the benign nature of the lesions and close monitoring of the
patients will continue.
CONCLUSION:
Papillomatosis is considered as a benign neoplasm
with an unpredictable course and with a low probability of
malignancy. We report of an unusual case of MEH persistent
for more than 30 years without regression and with
significant impact on a patient‘s quality of life.
Fig. 1. Papilomatosis of oral mucosa- hard palate and
gigngiva of upper jaw
http://www.journal-imab-bg.org
393
Fig. 2. Papilomatosis of oral mucosa- tongue, buccal
mucosa and gingiva of lower jaw
Fig. 4. Postoperative defect after electroexcision of
tumour
Fig. 3. Palatal tumour
Fig. 5. Postoperative defect covered with sutured
gauze, impregnated with Iodasept unguent.
REFERENCES:
1.Archard HO, Heck JW, Stanley
HR. Focal epithelial hyperplasia: an
unusual oral mucosal lesion found in
Indian children. Oral Surg Oral Med
Oral Pathol. 1965 Aug;20:201-12.
[PubMed]
2.Bombeccari GP, Pallotti F, Guzzi
G, Spadari F. Diode laser therapy for
Heck’s disease associated with HPV13
infection. J Eur Acad Dermatol
Venereol. 2009 Feb;23(2):197-8.
[PubMed] [CrossRef]
3.Blyumin M, Khachemoune A.
What is your diagnosis? Focal
epithelial hyperplasia (Heck disease).
Cutis. 2006 Apr;77(4):217, 233-4.
[PubMed]
4. Cohen PR, Hebert AA, AdlerStorthz K. Focal epithelial hyperplasia:
Heck disease. Pediatr Dermatol. 1993
Sep;10(3):245-51. [PubMed]
5. Garcia-Corona C, Vega-Memije
E, Mosqueda-Taylor A, YamamotoFurusho JK, Rodriguez-Carreon AA,
Ruiz-Morales JA, et al. Association of
HLA-DR4 (DRBI*0404) with human
394
http://www.journal-imab-bg.org
papilloma virus infection in patients
with focal epithelia hyperplasia. Arch
Dermatol. 2004 Oct;140(10):1227-31.
[PubMed] [CrossRef]
6. Greenspan D, de Villers EM.
Greenspan JS, deSouza YG, zur
Hausen H. Unusual HPV types in oral
warts in association with HIV infection.
J Oral Pathol. 1998 Nov;17(910):482-8. [PubMed]
7. Hall C, McCullough M, Angel
C, Manton D. Multifocal epithelial
hyperplasia: a case report of a family
/ J of IMAB. 2013, vol. 19, issue 4/
of Somalian descent living in Australia.
Oral Surg Oral Med Oral Pathol Oral
Radiol Endod. 2010 Jan;109(1):e2024. [PubMed] [CrossRef]
8.Hettwer KJ, Rodgers MS. Focal
epithelial hyperplasia (Heck’s disease)
in a Polynesian. Oral Surg Oral Med
Oral Pathol. 1966 Oct;22(4):466-70.
[PubMed]
9. Ledesma-Montes C, Garces-Ortiz
M, Hernandez-Guerrero JC. Clinicopathological and immunocytochemical
study of multifocal epithelial
hyperplasia. J Oral Maxillofac Surg.
2007 Nov;65(11): 2211-7. [PubMed]
[CrossRef]
10.March CJ. Multiple papillary
tumours of the labial, buccal and
glossal mucous membrane. Dental
Cosmos 1881;23:165.
11. Parkin DM, Almonte M, Bruni
L, Clifford G, Curado MP, Piñeros M.
Burden and Trends of Type-Specific
Human Papillomavirus Infections and
Related Diseases in the Latin America
and Caribbean Region, Vaccine. 2008
Aug 19;26 Suppl 11:L1-15. [PubMed]
[CrossRef]
12. Requena L, Requena C.
[Histopathology of the more common
viral skin infections]. [in Spanish]
Actas Dermosifiliogr. 2010 Apr;101(3):
201-16. [PubMed] [CrossRef]
13. Segura-Saint-Gerons R, Toro-
Rojas M, Ceballos-Salobreña A,
Aparicio-Soria JL, Fuentes-Vaamonde
H. Focal epithelial hyperplasia. A rare
disease in our area. Med Oral Patol
Oral Cir Bucal. 2005 Mar-Apr;10(2):
128-31. [PubMed]
14.Vera-Iglesias E, Garcia-Arpa M,
Sanchez-Caminero P, RomeroAguilera G, Cortina de la Calle P. Focal
epithelial
hyperplasia.
Actas
Dermosifiliogr. 2007 Nov;98(9):621-3.
[PubMed] [CrossRef]
15.Witkop CJ Jr, Niswander JD.
Focal epithelial hyperplasia in Central
and South American Indians and
Ladinos. Oral Surg Oral Med Oral
Pathol. 1965 Aug;20:213-7. [PubMed]
Address for correspondence:
dr Elitsa Deliverska,
Department of Oral and Maxillofacial surgery, Faculty of Dental Medicine,
1, Georgi Sofiyski blvd., 1431 Sofia, Bulgaria; tel.+359 888949740;
email: elitsadeliverska@yahoo.com,
/ J of IMAB. 2013, vol. 19, issue 4/
http://www.journal-imab-bg.org
395