Periodontal diseases and certain systemic disorders share similar genetic and/or environmental etiological factors, and affected patients may show manifestations of both diseases. Characterizing these diseases and the nature of the association between them could have important diagnostic value and therapeutic implications for patients

1. Dr. Nazia Yaseen
II MDS

15. • Shapira et al., 1991 - Periodontal treatment needs were obviously
higher in DS adults than in normal healthy adults
• Khocht A,2012 - higher levels of some subgingival bacterial species
and specific associations between certain subgingival bacterial species
and loss of periodontal attachment.
• Sakellari et al., 2001 - frequent recall program with 3-month period
could overcome inadequate supragingival plaque control and
subsequently altered subgingival environment
• Cheng et al., 2008 - satisfactory healing response on 21 DS individuals
treated by non-surgical mechanical periodontal therapy (followed by
monthly recalls) and the adjunctive use of chlorhexidine gel for
toothbrushing and chlorhexidine mouthwash twice daily.

18. Papillon Lefevre Syndrome
Etiology Main Clinical
Features
Other Sites
of
Involvemen
t
Periodontal
Involvement
Diagnosis Differential
Diagnosis
Treatment
Autosomal
recessive
trait.
 Gingiva are red,
swollen and
bleed easily.
 Severe
generalized
periodontitis with
deep periodontal
pockets and bone
loss are constant
features.
 hyperkeratosis of
palms and soles
 Psoriasiform
scaly red patches
Skin, always
present
(palms,
soles,
elbows,
knees,
dorsum of
the fingers
and toes)
Severe alveolar
bone loss,
exfoliation of
both deciduous
and permanent
teeth.
Clinical
Panaromic
radiography
Skin biopsy
 Generalized
aggressive
periodontitis
 Hypophosphatas
ia,
 Acatalasia
 Chediak-
Higashi
syndrome
 Langerhans cell
histiocytosis
 Glycogen
storage disease
Ib
 Congenital
neutropenia
 Cyclic
neutropenia
Dental plaque
control.
Good oral
hygiene
Aromatic
retinoids for skin
lesions

19. Cathepsin C gene in influencing
epithelial differentiation or
desquamation
The decrease in the chemotaxis
may be due to a cell defect in the
receptor located at the surface of
the neutrophils resulting in
deficiencies in the cellular adhesion
molecules deficiency of CD11
molecules
Aggregatibacter
actinomycetemcomitans was
detected in high frequency in the
patients with PLS and was
associated with the pronounced
bone loss of periodontal tissue in
these patients
Matrix metallo proteinase 8 in GCF
and atypical activity of
plasminogen system
PATHOGENESIS
OF PAPILLON
LEFEVRE
SYNDROME

27. Kostman Syndrome

28. kostman

29. Chronic Granulomatous Disease

30. Hyper ImmunoglobuminE

31. Cohen syndrome
Etiology General Features Periodontal
Manifestaion
Treatment
The etiology of this
disease is mutations
in the VPS13B
gene (frequently
called the COH1
gene
Low WBC,
The disease is
characterized by
intellectual
disability,
developmental
delay and a unique
physical
appearance
including narrow
hands and feet with
long, slender
fingers,truncal
obesity
Periodontitis Symptomatic

32. cohen

34. Kindler syndrome
Etiology Clinical
Features
Periodont
al
Findings
Other site
involveme
nt
Diagnosis Differenti
al
Diagnosis
Treatment
rare
autosomal
recessive
genetic
disorder1
caused by
mutation in
the KIND1
or
FERMT1
genes
Acral
blisters
Photosensi
tivity
Poikiloder
maSkin
atrophy
Gingival
fragility,or
al
ulcerations
Periodontit
is
Hands,skin Clinical
Genetic
susceptibili
ty
Dystrophic
epidermoly
sis bullosa
Weary
syndrome
Ehler
danlos
syndrome
Limiting
sun
exposure
and
antibiotics

37. Ehler Danlos syndrome

38. Etiology General Features Periodontal
Features
Other
sites
involvem
ent
Diagnosi
s
Differential
Diagnosis
Treatment
Genetic. It
is mainly
inherited as
an
autosomal
dominant
trait.
The skin
manifestations
include thin and
fragile skin.
hypermobility
haematomas.
scarring formation,
especially over the
knees, elbows and
chin. Joint
hypermobility
cardiovascular and
ocular abnormalities
mitral valve prolapse
Aggressive
Periodontiti
s
Oral
mucosa,
Skin,
Joints,
Heart
Clinical  Aggressive
periodontitis
associated
with other
genetic
disorder
 Marfan's
syndrome
 Cutis taxa
Symptomatic

41. Disorder Strength of
association
Mechanism Features Diagnosis
Angioedema Weak Inadequate control
of bradykinin
generation due to a
deficiency of
protease inhibitors
(C1-inhibitor)
and/or inadequate
degradation of
bradykinin into
inactive peptides
Serious and potentially
life-threatening attacks of
subcutaneous and
submucosal edemas of
upper airways, face,
abdomen, and extremities
Localized or generalized
severe periodontitis
Checking serum
C1 inhibitor or
ACE levels
based on
clinical
suspicion
Systemic lupus
erythematosus
Inconclusive Tissue destruction
may be due to
hyperactivation of
B and T
lymphocytes,
increased
production of IgG,
and production and
accumulation of
autoantibodies
Joint pain and swelling
affecting the fingers,
hands, wrists, and knees
Skin rash
Antinuclear
antibodies

43. Glycogen storage diseases

44. Etiology Clinical Features Periodontal
Findings
Other site
involvement
Diagnos
is
Differential
Diagnosis
Treatment
Genetic. It is
inherited as an
autosomal recessive
trait. This is a defect
of the microsomal
glucose-6-phosphate
translocase. The
disorder is
associated with
neutropenia and
neutrophil
dysfunction
Doll’s face, liver
and kidney
enlargement,
hypoglycaemia,
hyperlipidaemia,
growth retardation
and recurrent
bacterial infection
are common
Ulcers that
are usually
covered by a
whitish
pseudomemb
rane. Vaulted
palate and
recurrent oral
infection may
occurPeriodo
ntitis
associated
with genetic
disorder
Oral
mucosa.
frequent
Liver.
kidneys.
skeleton and
many other
organs
Clinical,
biopsy
 Congenit
al
neutropen
ia
 Cyclic
neutropen
ia
 Chediak-
Higashi
syndrome
 Hypopho
sphatasia

Acatalasi
a
Topical oral
antiseptics.
Systemic
treatment by
the
paediatrician.

47. Hypophosphatasia

48. Etiology General
Features
Periodontal
Features
Other
sites
involveme
nt
Diagnosis Differential
Diagnosis
Treatment
Genetic. It is
usually
inherited as
an autosomal
recessive
trait and
rarely as an
autosomal
dominant
trait
Skeletal
abnormalities
growth
retardation,
craniosynost
osis,
intracranial
hypertension
may occur
Early
exfoliation of
primary teeth
Very
common,
particularly
in the
childhood
form of the
disease
Skeleton,
skull, eyes
Panoramic
radiography.
Radiographic
examination of
the skeleton
and skull.
Measurement
of serum
alkaline
phosphatase
and blood and
urine phospho
ethanolamine.
 Localized
aggressive
periodontiti
s
 Acatalasia
 Papillon-
Letevre
syndrome
 Chediak-
Higashi
syndrome
 Glycogen
storage
disease Ib
Vit D
Parathhormo
ne
Phosphorous

53. Disorder Strength of
association
Mechanism Features Diagnosis
Osteoporosis Significant Increased bone
turnover leading
to net bone loss,
which can also
be associated
with other factors
(such as estrogen
level, vitamin D
and calcium
deficiency,
lifestyle and
behavioral
factors
Decrease in bone
mineral density
and weakening
of bone
microarchitecture
, leading to a
high risk for
bone fracture
No clear
association with
periodontitis
Clinical
Obesity Significant Possible
mechanisms
include an
impaired
immune response
and increased
production of
proinflammatory
cytokines
BMI ≥30
Increased risk
for periodontitis,
periodontal
progression, and
loss of
periodontal
attachment
Clinical
diagnosis

54. Acquired Neutropenia
An individual with an absolute neutrophil count (ANC) < 1500 cells/μl
CAUSES:
 Genetic
 Drug induced
 Viral infection

57. Inflammatory diseases that may be associated with loss of
periodontal tissue
Disorder Strength of
association
Mechanism Features Diagnosis
Epidermolysis
bullosa acquisita
Moderate Autoimmune disease
due to binding of
pathogenic
autoantibodies to
target antigens
Recurrent blister
formation of oral
cavity
Immunofluorescence
Inflammatory bowel
disease
Significant Autoimmune disease
in which a
hypersensitivity
immune response to
commensal gut
bacteria
Abdominal pain,
fever, diarrhea, and
weight loss
Colonoscopy
Arthritis Significant Autoimmune disease Joint pain, swelling,
stiffness, redness,
and limited motion
Clinical examination

58. Other systemic disorders that may contribute to the loss of
periodontal tissue by influencing periodontal inflammation
Disorder Strength of
association
Mechanism Features Diagnosis
Emotional stress
and depression
Weak Activation of the
limbic-
hypothalamic
pituitary-adrenal
axis leads to the
release of
neuroendocrine
peptides and
hormones that
modulate the
immune response
Changes in
behavior, mood,
and physiological
markers
Psychological
evaluation
Hypertension Inconclusive Undetermined Chronic status of
high blood
pressure
Physical
examination

59. LANGERHANS CELL HISTIOCYTOSIS
Eosinophilic
granuloma that is
a localized benign
form of the
disorder, usually
characterized by
solitary or multiple
bone lesions and
soft tissue lesions
as well
Hand-Schuller-
Christian disease
that is a chronic
disseminated form
involving
primarily bone,
soft tissue and
viscera.
Lettere-Siwe
disease
that is an acute
disseminated
form involving
skin, viscera,
bone marrow.

60. Etiology General Features Periodontal
Features
Other
sites
involveme
nt
Diagnosis Differential
Diagnosis
Treatment
Heterogene
ous disorder
characterize
d by
proliferatio
n of
Langerhans
cells.
Jaw bone
destruction Other
bone involvement,
exophthalmos,
diabetes insipidus,
skin rash, otitis
media,
hepatomegaly,
splenomegaly,
lymphadenopathy
More than 50 % of
all cases of the
Langerhans cell
histiocytosis are
seen in patients
under 10 years of
age
The gingival
lesions
present as
localized or
multiple
atypical
ulcerations,
usually
associated
with bone
destruction
and teeth
loosening
Oral
mucosa,
jaws
Bones,
bone
marrow,
skin,
visceral
Clinical
,Biopsy and
histopatholo
gical
examination
.
Immunohist
ochemical
examination
s
Radiographi
c
examination
.
 Necrotizing
ulcerative
gingivitis and
periodontitis
 Aggressive
periodontitis
 Traumatic
lesions on the
gingiva
 Hypophospha
tasia
 acatalasia
 Haematologi
cal disorders
 Eosinophilic
ulcer
Radiation
Corticosteroid
therapy

64. Barret, A.P., 1984. Gingival Lesions in Leukemia. J Periodontol, 55, pp.585-588

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