Diseases of skin 4/ dental implant courses

DISEASES
OF
THE SKIN
HEREDITARY ECTODERMAL
DYSPLASIA, PSORIASIS
INDIAN DENTAL ACADEMY
LEADER IN CONTINUING DENTAL
EDUCATION
www.indiandentalacademy.com

LEARNING OBJECTIVES
At the end of the lecture student should beAt the end of the lecture student should be
able toable to describe thethe
 Clinical features, Oral manifestations,&
Histopathological features of Hereditary ectodermal
dysplasia ,Psoriasis, Epidermolysis bullosa,
Incontinentia pigmenti

HEREDITARY
ECTODERMAL DYSPLASIA
•Hereditary ectodermal dysplasia is an
inherited X-linked recessive
disorder characterized by the
defective formation of ectodermal
structures of the body (e.g. skin, teeth,
nails, sweat glands, sebaceous glands
and hair follicles, etc.)

CLINICAL FEATURES
 The three most outstanding features are-
1. Hypohydrosis (lack of sweating),
2. Hypotrichosis (absence of hair) and
3. hypodontia (absence of teeth).
 Occurs more frequently among males than
females.

 Soft, dry and smooth skin
with little or no tendency
for sweating
 May have an unexplained
fever and they cannot
endure warm temperatures
 The hair over the scalp and
eyebrows are fine, scanty
and blond, and sebaceous
glands are also absent
(asteatosis)

 Complete or partial
anodontia occurs that
involves both deciduous as
well as the permanent
dentition (only the canines
are often present).
 The teeth which are
present, are often small
and conical in shape.
 Xerostomia is a constant
feature which occurs due to
the decreased salivary
secretion.

 The patient may also have rhinitis, sinusitis and
pharyngitis etc, with dysphagia and hoarseness
of voice
 Typical facial appearance characterized by
depressed nasal bridge, frontal bossing
and protuberant lips
 High palatal arch & cleft palate

TREATMENT
 The typical clinical manifestations always confirm
the diagnosis of this disease and there is no specific
treatment for it.
 Artificial dentures (with soft liners) are constructed
and are changed from time to time to cope up with
the growth of the jaws and artificial saliva is given to
keep the mouth moist.

PSORIASIS
Psoriasis is a self-limiting, chronic
inflammatory dermatological disease of
unknown etiology.
Some investigators believe that its
occurrence is genetically determined

Clinical Features
 Predominantly in the 2nd
& 3rd
decade of life.
 No sex predilection.
 Painless, dry, white scaly patches, that appear on
the skin over the elbows, knees, scalp, chest &
face, etc.
 The patches are well-circumscribed &
erythematous, few lesions can even produce sterile
pustules.

 The lesions enlarge at the periphery & the
disease often shows periods of remissions and
exacerbations.
 Mental anxiety or stress often increases the
severity of the disease.
 "Auspitz's sign“-If the deep scales on the
surface of the lesion are removed, one or two
tiny bleeding points are often disclosed.

 Oral lesions of psoriasis- Erythematous patches
with white scaly surfaces over the lips, palate,
gingiva & cheek, etc.
 Some lesions may appear as well-defined,
grayish-white or yellowish-white patches. In
some cases these oral lesions may resemble
'geographic tongue'.
 Sometimes psoriasis may be accompanied by
arthritis-Psoriatic Arthritis

HISTOPATHOLOGY
 The oral epithelium shows
atrophy with hyper -
parakeratosis, absence of
granular cell layer and
elongation or clubbing of the
rete pegs.
 Intraepithelial micro abscess
formation (abscess of Monro)
is an important histological
finding of psoriasis.

 There is always an
increased mitotic activity
seen in the psoriatic skin
or mucous membrane.
 Thesubepithelial
connective tissue shows
mild lymphocytic or
histiocytic cell infiltrations.

EPIDERMOLYSIS BULLOSA
It is a generalized desquamating condition
of the skin & mucosa which is often
associated with scarring, contractures &
dental defects.
Separation of the epithelium from the
underlying connective tissue with
formation of large blisters, which often
heal with extensive & often immobilizing
scar formation.

Two broad categories:
 1. Hereditary epidermolysis bullosa include
epidermolysis bullosa simplex, epidermolysis
bullosa dystrophic and junctional epidermolysis
bullosa.
 2. Acquired epidermolysis bullosa. There is
only a single form of named as epidermolysis
bullosa aquisita.
 This acquired from of the disease may be
associated with multiple myeloma, diabetes
mellitus, tuberculosis, amyloidosis and crahn's
disease, etc.

CLINICAL FEATURES
 Characterized by the formation of multiple
vesicles or bullae on the pressure areas of
the skin (i.e. elbows and knees).
 Dystrophic type-most severe form & may
cause death secondary to septicemia.
 The lesions rupture & leave raw, painful ulcers,
which heal up with scarring.

 The healing of skin lesions cause scarring
with pigmentation or depigmentation of
the area.
 Nails often shed or exfoliate due to
formation of blisters in the nail beds.
 The hereditary form
 Very severe in nature & in infancy or
early childhood
o Acquired form
 Common during adulthood only.

 May also exhibit stunted
growth, mental retardation and
ectodermal dysplasia, etc.
 May have alopecia & claw-
like hands due to repeated
scarring and contractures.
 Many such patients die during
childhood.

ORAL MANIFESTATION
 Oral lesions are particularly common &
severe in relation to the hereditary forms
of the disease.
 Rapidly developing, multiple, fragile &
hemorrhagic blisters or bullae in the
areas of trauma (particularly in the
palate).

 The lesions rupture soon & leave painful
ulcers, which later on heal by
scarring.
 Repeated blistering & scarring around the
oral cavity result in decreased mouth
opening, ankyloglossia & loss of vestibular
sulci.

 May develop perioral & perinasal crusted &
hemorrhagic granular lesions.
 Sometimes oral lesions may transform into
squamous cell carcinoma.
 Hypoplastic pitted enamel of the molar
teeth
 Delayed eruption of tooth, increased caries
susceptibility & increased periodontal diseases.

HISTOPATHOLOGY
 Destruction of the basal or the
suprabasal layers of the oral
epithelium, resulting in the
formation of vesicles or bullae.
 Bullae formation may also be
seen within enamel organ of the
developing tooth germ.

 Disease type: Level at which bullae
forms.
1. Simplex type: intraepithelial bullae.
2. Junctional type: at the level of lamina lucida,
3. Dystrophic type: at the level of lamina densa,
 Treatment: Systemic steroid therapy.
Immunosuppressive drug therapy . Avoidance of
trauma

INCONTINENTIA PIGMENTI
It is a serious type of inherited
genodermatosis, which is transmitted
as a sex-linked dominant trait.

CLINICAL FEATURES
 Mostly during infancy & although it is more
common among females, it is often lethal among
males.
 Clinical manifestation begins to appear shortly
after birth & is characterized by slate- grey
pigmentation of the skin with formation of vesicles
or bullae over the trunk & limbs.

HISTOPATHOLOGY
 During the verrucous stage of the disease,
intra-epithelial vesicle formation is often
seen with accumulation of large number of
eosino-phils.
 Dermal or submucosal accumulations of
macrophages & melanin granules are also
seen. White areas display
hyperorthokeratosis or parakeratosis with
acanthosis.
 Individual cell keratinization is also
sometimes seen.
 Treatment -No specific treatment is
available

SUMMARY
Introduction, Clinical features, Oral
manifestations,& Histopathological features of
 Hereditary ectodermal dysplasia
 Psoriasis
 Epidermolysis bullosa
 Incontinentia pigmenti

BIBLIOGRAPHY
 Text book of oral pathology Shafer's, 5 & 6th
edition
 Oral & Maxillofacial Pathology A Rationale for
Diagnosis & Treatment. R E Marx 1st
edition
 Color Atlas of Oral Diseases Cawson, R. 2nd
& 5th
edition
 Oral and Maxillofacial Pathology Neville, Brad
W. 2nd
 Lucas’s Pathology Of Tumor’s of the Oral Tissues
 Robbins Basic Pathology, Kumar V, A Fausto, 8th
edition
 Lever’s Histopathology of the skin, David Elder
8th
edition

Diseases of skin 4/ dental implant courses

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