CONCISE REVIEW
A Concise Update on the Syndromes Affecting the Periodontium
Suchetha A1, Darshan B Mundinamane1, Sharadha Jaganath1, Bharwani Ashit G1
1 Department of Periodontics
D A Pandu Memorial
R V Dental College
Bangalore, Karnataka, India
ABSTRACT
Journal of Dental Sciences and Research
Vol. 2, Issue 2, Pages 1-5
Syndromes affecting the periodontium are not very common; hence they are missed on routine
clinical diagnosis. The knowledge of these syndromes is essential as they can influence the
prognosis and management of periodontal disease. The aim of this review is to address how
syndromes affecting the systemic health of an individual can also affect the periodontal health.
Keywords: Syndromes, systemic conditions, periodontal manifestations
INTRODUCTION
]
Syndromes Causing Red Lesions in the Gingiva
Syndromes are systemic conditions with widespread
manifestation throughout the body [1]. This review
focuses on the syndromes affecting the periodontium
with highlights on the periodontal manifestations of each
syndrome.
Klippel-Trenauny-Weber syndrome (OMIM 14900) and
Sturge-Webber syndrome (encephalotrigeminal
angiomatosis) (OMIM 176920) are two syndromes
which manifest as capillary haemangiomas on the
gingiva. They can also lead to localized gingival
enlargement and gingival bleeding[5]. The lesions have a
bright red or purple colour and are usually flat. Dentists
must be careful during tooth extractions and periodontal
surgery so as to avoid bleeding complications[4].
A syndrome is a combination of symptoms resulting from
a single cause or so commonly occurring as to constitute
a distinct clinical entity[2]. A group of symptoms those
together are characteristic of a specific disorder, disease,
or the like constitute a syndrome[3].
SYNDROMES AFFECTING THE
PERIODONTIUM
]
Syndromes Causing White Lesions in the Gingiva
Focal palmoplantar and oral mucosa hyperkeratosis
syndrome (OMIM (Online Mendelian Inheritance in
Man) 148730) or hyperkeratosis and attached gingival
hyperkeratosis is an unusual genetic disorder (autosomal
dominant) characterized by oral mucosal and dermal
hyperkeratosis. Marked white hyperkeratosis of the
attached gingiva, presenting clinically as leukoplakia is a
consistent finding[4].
Address for correspondence:
Dr Sharadha Jaganath
E-mail: [email protected]
Access this article online
Website: http://www.ssdctumkur.org/jdsr.php.
36
Melkersson – Rosenthal syndrome (OMIM- 15590)
consists of the triad of recurrent facial swelling, facial
paralysis and fissured tongue. Gingival lesions are
present as irregular, oedematous swelling, slightly
erythematous, affecting mainly the anterior interdental
papillae and free and attached gingiva.
Sjogren's syndrome (OMIM 270510) is a relatively
common, chronic autoimmune disorder in which chronic
gingivitis localized or generalized due to xerostomia
and/or Candida infection may occur. Rarely, alveolar
bone loss can also be seen[5]. The oral mucosa is reddish,
dry, smooth and shiny.4 Gingival lesions in OslerRendu-Weber syndrome/hereditary haemorrhagic
telangiectasias (OMIM 187300) appear as small,
localized or multiple, bright red macules that
characteristically disappear when pressure is applied.5
Gingival recurrent bleeding, usually, during or after
tooth brushing, is frequent[4].
]
Syndromes causing gingival bleeding
Sturge-Webber syndrome, Klippel-Trenaunay-Weber
syndrome, Chediak-Higashi syndrome are the three
Vol. 2, Issue 2, September 2011
syndromes that are thought to predispose to gingival
bleeding. The former two syndromes cause gingival
bleeding because of vascular malformations, the latter
causes gingival bleeding secondary to defects in
platelets[4].
]
Syndromes Causing Defects in the Gingivolabial
Fold
In Orofacial syndrome type I (OMIM 311200) the
gingival involvement is characterized by multiple
hyperplastic fibrous bands traversing the gingivolabial
and gingivobuccal fold, hypertrophy and shortening of
the frenulum of the lips and the tongue. These may result
in hyperplastic gingivitis and gingival recession[4]. Ellis
van Crevald syndrome/ chondroectodermal dysplasia
(OMIM 225500) is characterized by the fusion of the
middle portion of the upper lip to the gingiva leading to
the disappearance of the mucolabial fold and multiple
frenula are the most characteristic oral manifestation[5].
]
Syndromes Causing Ulcers and Erosions in the
Gingiva
Bechet's syndrome (OMIM 109650), Rieter's
syndrome[4] and Myelodysplastic Syndrome[6] are known
to present with ulcers on the attached gingiva. The
lesions can vary from non specific erythema to recurrent
painful ulcers. Bleeding from the lesions can be present
at times.
Although aphthous ulceration on the oral mucosal tissues
is common in the PFAPA syndrome (periodic fever,
aphthous stomatitis, pharyngitis and adenopathy) /
Marshall Syndrome, gingival involvement is
uncommon[7].
]
Syndromes Causing Pigmentation in the Gingiva
Peutz-Jegher's syndrome (OMIM 175200) and
Albright's syndrome (OMIM 174800) manifest with
pigmented spots on the face, oral cavity and sometimes,
hands and feet[4, 5].
]
Syndromes Causing Localized Lumps or
Swellings in the Gingiva
The two syndromes that present with localized lumps in
the gingiva include Gardener's syndrome in which the
lesions are multiple osteomas and Turner's syndrome, in
which the lesions are tori in the mandible[4, 5].
Puretic-Drescher syndrome (OMIM 228600) [ 4 ] ,
Rutherford syndrome[9], Ramon Syndrome (OMIM
266270) [8].
Hurler's syndrome (OMIM 2607014) /
Mucopolysaccharidosis I (MPS I) is a rare and most
common form of Mucopolysaccharide metabolic
disorders. The main feature is gingival overgrowth that
may fully or partially cover the crowns of the teeth,
particularly in the anterior area of the maxilla. The
overgrowth is mostly the result of microbial dental
plaque, mouth breathing and deposition of dermatan
sulphate and heparan sulphate in the gingival tissue[9].
The third type of generalized gingival swellings can be of
the nodular or papillomatous type. These lesions can be
seen in Focal Dermal Hypoplasia syndrome/ GoltzGorlin syndrome (OMIM 305600), Bourneville-Pringle
s y n d r o m e ( O M I M 1 9 11 0 0 ) a n d C o w d e n ' s
syndrome/multiple hamartoma syndrome.
]
Syndromes Affecting the Periodontal Ligament
Scleroderma (OMIM 181750) is a connective tissue
disorder the characteristic feature of which is an
inflammatory, vascular and fibrotic change in the skin
and other structures. Scleroderma is associated with two
syndromes- CREST syndrome (Calcinosis cuti,
Raynaud's disease, esophageal strictures, sclerodactyly
and telangiectasias of the skin) and ThibiergeWissenbach syndrome[9]. The characteristic feature of
scleroderma is the uniform widening of the periodontal
ligament space at the expense of the alveolar bone.
]
Syndromes Causing Periodontal Destruction
The two categories of disorders causing periodontal
destruction can be grouped as Neutrophil defects and
Collagen disorders.
COLLAGEN DISORDERS
The Ehlers–Danlos syndromes are a heterogeneous
group of inherited connective tissue disorders
characterized clinically by skin fragility, skin
hyperextensibility, joint hypermobility, and excessive
bruising. Poor wound healing is a component of a number
of subtypes of Ehlers–Danlos syndrome, and severe,
early-onset periodontitis has been associated with two
subtypes: Ehlers–Danlos syndrome type 4 and
Ehlers–Danlos syndrome type[8, 10].
]
Syndromes Causing Generalized Lumps or
Swellings in the Gingiva
The syndromes that can be grouped in this category
include those which present as familial gingival
fibromatosis. These syndromes include Laband
syndrome (OMIM 13550)[4], Cross syndrome[8], Murray-
NEUTROPHIL DEFECTS
Defects in the functions of neutrophils or a marked
decrease in the number of neutrophils capable of
responding to the site of infection may result in varying
degrees of susceptibility to infection.
37
Journal of Dental Sciences and Research
a. Quantitative Neutrophil Defects
A relative deficiency in neutrophil number can
dramatically increase susceptibility to infectious
diseases. Quantitative defects can be at the bone marrow
like in Kostman syndrome (OMIM 610738) and Felty's
syndrome or at the periphery as in Lazy leukocyte
syndrome. Other syndromes that are associated with
decreased neutrophil counts and periodontal destruction
include Herman sky–Pudlak syndrome and
Shwachman–Diamond syndrome. All syndromes
present with wide spread and early periodontal tissue
destruction.
b. Qualitative Neutrophil Defects
These can be defects in rolling and adhesion (Leukocyte
adhesion deficiency syndrome ), defects in migration
and chemotaxis (Hyperimmunoglobulin E syndrome,
lazy leukocyte syndrome, Papillon-Lefvre syndrome,
Down's syndrome, Kindler syndrome) and defects in
phagocytosis and intracellular killing (Chediak Higashi
syndrome)
Leukocyte adhesion deficiency syndrome (LAD) is of
two sub types I and II. LAD-I is a disorder that involves a
deficiency in three membrane integrins- CD18/CD11a,
CD18/CD11b, and CD18/CD11c which bind to
leukocytes and to endothelium via intercellular adhesion
molecules (ICAM). The neutrophil defect in LAD-II is of
the sialyl-Lewis x glycoprotein (CD15s), which allows
neutrophils to attach to selectins (CD62E) on the
endothelial surface. The end result is that the neutrophils
are unable to migrate extravascularly. Acute gingival
inflammation of the primary and permanent dentition,
gingival proliferation, gingival recession, mobility of
teeth and pathological migration are common clinical
features.
Hyperimmunoglobulin E syndrome is a multisystem
disorder inherited as an autosomal dominant trait that
affects the dentition, the skeleton, connective tissues,
and immune system. Over-retention of primary teeth in
contrast with the early loss of primary teeth due to
periodontitis seen in other disorders of host defenses, is
surprising.
The Papillon-Lefevre syndrome (OMIM 245000) is
described as a diffuse palmoplantar keratosis associated
with aggressive periodontitis of both primary and
permanent dentitions. A particular form of PapillonLefevre syndrome has been named the Haim-Munk
syndrome, and is also associated with early periodontal
destruction[10, 11].
Down's syndrome was named after the English physician
who in 1866 characterized the appearance and behavior
of these patients. Gingival hyperplasia can occur
secondarily due to mouth breathing, poor hygiene, and
local irritating factors. The gingivitis frequently
38
progresses to generalized early periodontitis, which
commences in the deciduous dentition and continues to
affect the permanent dentition[12].
Another syndrome with severe gingivitis, ulcerations of
the tongue and buccal mucosa, and early onset
periodontitis leading to premature loss of both deciduous
and permanent dentitions is the Chediak-Higashi
syndrome (OMIM 214500). Bleeding problems arise
because of organelle abnormalities within platelets that
inhibit normal clot formation.
Other syndromes with qualitative neutrophil defects that
could predispose to periodontal destruction include
Kindler syndrome and Hypotrichosis osteolysis
periodontitis palmoplantar keratoderma syndrome
(OMIM 607658)[11].
CONCLUSION:
It is well established that many systemic conditions
express their effects on the periodontium. Future
epidemiological studies designed to assess the role of
systemic conditions and disorders in periodontal disease
are needed, particularly to refine experimental design
and data analysis; to identify gaps in knowledge with
respect to mechanisms of factors known to play a role in
increasing susceptibility to periodontal disease; and to
address gaps in knowledge of the correlation with the
systemic conditions suspected of being related to
periodontal disease.
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