Skin disease including lichen planus, pamphigus, pemphigoid

Dental students UG level

1. SUBMITTED BY RUPALI,SHIKHA,ASHU,RICHA

2. DEFINITIONS

> Macule- circumscribed, flat lesion with color change up to 1 cmin
size that is not palpable.

> Patch- flat lesion more than 1 cm in size.

> PAPULE - A circumscribed, elevated, solid lesion that is less than 10
mm in diameter

> NODULE - A palpable, solid lesion that is greater than 10 mm in
diameter.

> VESICLE- Superficial blister,5mm or less In diameter, usually filled
with clear fluid.

> Bulla- Large blister, greater than 5mm diameter.

3. » Erosion is characterized by the partial loss of the epithelium, with the
basement membrane left intact.

> Pustule- blister filled with purulent exudate.

» Ulcer-Loss of both epidermis and dermis.

4. VESICULOBULOUS LESION a

Vesiculobullous (VB) diseases are a distinct group of oral
disorders characterized by the formation of vesicles or
bullae. It is uncommon to see vesicles or bullae intraorally
as they soon rupture, leaving erosions or ulcers.

Fitzpatrick classification - Based on specific
separation according to the anatomical plane

5. According to separation at
intraepithelial level

+ Erythema + Pemphigus + Familial Pemphigus
multiforme vulgaris benign foliaceous

+ Toxic epidermal + Pemphigus pemphigus Pemphigus
necrolysis vegetans Herpes erythematosu

+ Lichen planus = Caries simplex virus s

+ Lupus
erythematosus

+ Epidermolysis
bullosa simplex

disease infection Frictional
Herpes blisters
zoster and Bullous
varicella impetigo
Eczematous

dermatitis

6. According to separation at
dermo-epidermal junction

+ Bullous pemphigoid
+ Cicatricial pemphigoid

| - Epidermolysis bullosa
acquisita
+ Epidermolysis bullosa

« Epidermolysis bullosa
junctional

+ Dermatitis
herpetiformis

dystrophica
« Linear IgA dermatosis
- Bullous systemic lupus
erythematosus (SLE)

7. ECTODERMAL DYSPLASIA B

» Synonyms: Hereditary ectodermal dysplasia, Ectodermal

dysp

asia syndrome.

> Ectodermal dysplasia syndrome (EDS) is a large, heterogeneous
group of inherited disorders, the manifestations can be seen in
more than one ectodermal derivatives.These tissues primarily are
the skin, hair, nails, eccrine glands, and teeth.

> The current classification of ectodermal dysplasia (ED) is based

onc

inical features.

» The most common syndromes within this group are

a)H

ypohidrotic (anhidrotic) ED and

b)Hidrotic ED

8. > Several EDSs may manifest in association with midfacial
defects, mainly cleft lip and palate.

» Etiology: Ectodermal dysplasia syndrome results from
aberrant development of ectodermal derivatives in early
enr life. Different genes are involved in different types
fe}

9. CLINICAL FEATURES

1.Some EDS types are mild, while others are
devastating.

2. Most often in whites.

3. X-linked hypohidrotic ED affects only in
males.Female act as carriers with little or no
signs of the condition

4. The remaining EDSs have no gender
predilection

5.Dental hair, nail anomalies

6.Dry mouth , teeth Show abnormal
morphogenesis or may be absent.
7.Eccrine glands or mucous glands may be

absent in upper respiratory
A tract, brochi,esophagus
SSS 8.Cleft lip and palate.

e uenthospitalisations 2 Vision or hearing defect, missing fingers and
toes.

pped jaw
Few misshapen teeth

10. HYPOHYDROTIC SYNDROME a

«also known as anhidrotic ED ,Christ-Siemens Touraine.

Most common phenotype in this group Usually inherited as an X-linked
recessive trait; autosomal recessive and autosomal dominant forms
have been reported but are rare.

It is characterized by several defects

a) Hypohidrosis. c)Onychodysplasia
b)Hypotrichosis. d)Anomalous Dentition
Typical facies are characterized by:.

Frontal Bossing,Sunken Cheeks

Saddle Nose,thick everted lips

Large low set ears
Wrinkled, Hyper pigmented Skin Around the eyes

11. ORAL MANIFESTATION

«hypodontia or complete anodontia.
«conical or peg lateral tooth.

«delayed eruption of permanent teeth.
«high palatal arch or cleft palate
«hypoplastic xerostomia

«protuberant lips,dry or cracked lips.
hypoplasia of mucous glands.

12. HIDROTIC ED (CLOUSTON
SYNDROME)

> Autosomal dominant manner.
> Clinical features include:

1.Nail dystrophy Nails are thickened and discolored;
persistent paronychial infections are frequent.

2.Hair defects - Scalp hair is very sparse, fine, and brittle.
Eyebrows are thinned or absent.

3.Palmoplantar dyskeratosis.
4.Patients have normal facies, normal sweating
5.No specific dental defect is seen.

13. HISTOLOGICAL FINDINGS

«reduction in the number of sweat glands, hair follicles, and

sebaceous glands

«In hypohidrotic EDS, the epidermis is thin and flattened.
*Eccrine sweat glands are few or poorly developed or are

very rudimentary.

«Salivary glands may show ectasia of ducts and inflammatory

changes

TREATMENT

‘There is no treatment for the condition Affected
individuals with dental defects could be subjected to
early dental evaluation and intervention beginning with
dentures as early as two years.

14. PSORIASIS

> Noncontagious chronic skin disorder

» Most commonly appears as inflamed, edematous skin lesions covered
with a silvery white scale. The most common type of psoriasis is plaque
psoriasis and is characterized by patches on the scalp, trunk, and limbs.

> Etiology

¢ The cause of psoriasis is unknown.

«Genetic predisposition has strong association with HLA Cwé and B57.
« some evidence suggest that psoriasis is an autoimmune disease.

«associated with increased activity of T-cells in underlying skin.
» stress-related disease.

15. PATHOGENESIS

Hyperactivity of T-cells
Epidermis infiltration & keratinocyte proliferation
Deregulated inflammatory res;
ge production of tokines

uperficial blood vessels dilated and vascular engorgement
Epidermal hyperplasia & improper cell maturation

Fails to release adequate lipid which lead to flaking,
scaling presentation of psoriasis lesion

16. » Clinical Features B

«Lesions on the skin is characterized by the occurrence of small,
sharply delineated, dry papules, each covered by a delicate
silvery scale which has been described as resembling a thin layer
of mica

« If the deep scales are removed, one or more tiny bleeding
points are disclosed - Auspitz's sign

«After removal of the scale the surface of the skin is red and dusk’
in appearance.

17. he cutaneous lesions are painless and seldom pruritic

«The disease commences with the appearance of a few small papules,
which gradually increase in size.

he papules enlarge at the periphery and tend to become slightly
infiltrating and elevated, smaller lesions coalescing to form large
plaques of irregular outline.

«They are roughly symmetrical and are most frequently grouped on the
extensor surfaces of the extremities, particularly the elbows and knees,
the scalp, back and chest, face and abdomen.

he disease may remain static for a long time, progresses slowly.
«severe in winter>summer ;as a result of increased exposure to
ultraviolet light

*Mental anxiety or stress - increase the severity of the disease «Psoriasis
is uncommon in children It most frequently arises in the second and
third decades of life. The median age at onset is 28 years. Psoriasis is
slightly more common in women.

18. Oral Manifestations: o

-Psoriatic involvement of oral mucosa extremely rare

* lesions have been reported on the lips, buccal mucosa, palate,
gingiva and floor of the mouth Clinically, they are described as
gray or yellowish-white plaques; as silvery white, scaly lesions with
an erythematous base

«Oral lesions have exhibited all histologic features of psoriasis and
in some instances have been identical with the coexisting skin
lesions.

Psoriaisiform lesions

a) Psoriasis. C)Benign migratory glosittis

b) Reiter's syndrome d) Geographic tongue

19. HISTOLOGICAL features

Parakeratosis
Absence of the stratum granulosum
Elongation and clubbing of the rete pegs

The epithelium over the connective tissue papillae is thinned, and it is from
these points that bleeding occurs when the scales are peeled off. Tortuous,
dilated capillaries extending high in the papillae are prominent.

Intraepithelial microabscesses (Monro's abscesses)

Treatment:

UV-A light, psoralen plus UV-A light (PUVA), retinoids (e.g., isotretinoin,
acitretin), methotrexate (particularly for arthritis)

20. Figure 19-8. Psoriasis
Photomicrograph of skin psoriasis exhibits the pathognomonic features of the
disease (Courtesy of Dr Mary Sebury Stone).

21. ORAL LICHEN PLANUS

>
>
>

El

>

>

Can affec

It can cau:
he bucca

precedes

t is common mucocutaneous disease.

either the skin or mucosa or both.

se bilateral white striations, papules, or plaques on
mucosa, tongue, and gingivae. The involvement of

the oral mucous membrane is so frequent and accompanies or

he appearance of lesions on the skin and genital

mucous membrane

IOLOGY-

t is a T-cel-mediated autoimmune disease in which cytotoxic

CD8+ T-cel

peptide.

Is trigger the apoptosis of oral epithelial cells.

he precise cause of OLP is unknown,although it may self

22. CD8+ lesional T-cells may recognize an antigen associated with
the MHC class | on keratinocytes.

After antigen recognition and activation, CD8+ cytotoxic T-cells
may trigger keratinocyte apoptosis.

|

Activated CD8+ T-cells may release cytokines that attract
additional lymphocytes into the developing lesion.

The expression or unmasking of the lichen planus antigen:

1.May be induced by drugs (lichenoid drug reaction)

2.Contact allergens in dental restorative materials toothpastes (contact
hypersensitivity reaction)

3.Mechanical trauma (Koebner phenomenon)

4 Niral infection.

23. Clinical Features:

oF: M- 1.4:

«it predominantly occurs in adults
older than 40 years

Skin lesions - appear as small,
angular, flat-topped papules only
a few millimeter in diameter
gradually coalesce into larger
plaques, each of which is covered
by a fine, glistening scale.

«Early in the course of the disease
the lesions appear red purple or
violaceous hue brownish color
develops.

«ts surface is covered by
characteristic, very fine grayish-
white lines, called Wickham's
striae.

GRINSPAN SYNDROME

24. 6 P’s of lichen planus

Pruritic: Itchy

Polygonal: The rash has many sharp angles
Planar: The top is flat

Purple

Papules: Bumps that are purple

Plaques: Raised, discolored patches

«usually distributed in bilaterally symmetrical pattern, most
often flexor surfaces of wrist , inner aspect of knees, chest
¿abdomen

«Scalp involvement

25. ORAL MANIFESTATION

> In the oral cavity - lesions consisting of radiating white or gray,
velvety, thread-like papules in a linear, annular or retiform
arrangement forming typical lacy, reticular patches, rings and
streaks over the buccal mucosa

» A tiny white elevated dot is frequently present at the
intersection of the white lines, known here also as the striae of
Wickham.

» Buccal mucosa most common site followed by tongue, lips,
gingiva, floor of mouth and palate.

» Patients will complain of a burning sensation in the involved
areas.

26. Types of lichen planus

6 types

1.Plaque like.

2.Papular. -Painless and white keratotic
3.Reticular

4.Atrophic

va ~ Painful, burning sensation
6.Bullous

27. Ulcerative Atrophic Bullous

28. HISTOPATHOLOGICAL FEATURES u

+ Orthokeratosis / Parakeratosis

« Acanthosis

+ Saw-toothed reteridges

+ Hydropic degeneration of basal cell layer

« Degenerating keratinocytes - Civatte / Colloid /Hyaline bodies
+ Intense band-like subepithelial infiltrate cheifly T- lymphocytes

«degeneration of nasal keratinocytes and description of hemi
desmosomes, filament,fibrils weaken the epithelial-connective tissue
interface result in maxjoseph spaces

29. Showing hyperkeratosis, saw-toothed
reteridges and band like subepithelial
infiltrate of lymphocytes

Showing migration of lymphocytes into
epithelium with degeneration of basal
cell layer

30. Malignant Transformation

«The overall frequency of malignant transformation is low,
varying between 0.3 and 3%

«The forms that more commonly undergo malignant
transformation are the erosive and atrophic forms.

Treatment

At present there is no cure, although various agents have
been tried. AS it is an autoimmune mediated condition,
corticosteroids are recommended.

31. LICHENOID REACTION

> Lichenoid reaction is an oral mucosal condition that is clinically and
histologically indistinguishable from OLP except for identifiable causes.

ETIOLOGY-

«dental restoration

«graft vs host reaction

«Flavoring agents, tobacco chewing

¢drugs- antimalarial, NSAIDS, antiviral during HIV infection.
CLINICAL FEATURES

«similar to lichen planus except atypical location and
absence of bilateral Occurence.

32. > HISTOLOGIC FEATURES.

«AlThough, histologically, LR has superficial resemblance to lichen
planus, there are notable differences.

Only focal parakeratosis or focal interuption of the granular layer
is seen.

he inflammatory infiltrate is diffuse and extends deeper into the
lamina propria unlike the sharp band of infiltrate seen in lichen
planus.

nflammatory infiltrate consists of plasma cells and eosinophils in
dition to lymphocytes.

+ Increased numbers of colloid or Civatte bodies may be present
in LR in the granular and keratinized later.

Lichenoid dysplasia seen and has marked malignant
predisposition.

Q

33. > Treatment and prognosis

* Identification and elimination of the triggering factors
play a major role in the management of LR.

« Lichenoid lesions can take many months or longer to
resolve.

«The malignant transformation rate is higher in oral
lichenoid lesions which do not have all the typical clinical
and histologic features of OLP.

35. «Erythema Multiforme is an acute self limiting
dermatitis characterized by distinctive clinical
eruption manifested as the iris or target lesion.
It has various morphological appearance such
as macule, papule, bullae and crust, hence
the name '‘Multiforme’

36. It exhibits the spectrum of severity ranging from:

EM minor:- localized eruption of the skin with mild or no mucosal
involvement.

2. EM major:- severe form, mucosal erosions of raised atypical target
lesions. These are usually located on the extremities and/or on the.

3. Stevens-Johnson syndrome (SJS) severe form, mucosal erosions +
widespread distribution of flat atypical targets. The lesions may be
present on trunk,face and extremities

37. Etiopathogenesis

» Not fully understood but is probably an immunologically
mediated process..

> May be precipitated by some bacterial, fungal and viral
infections.

> Herpes Simplex Virus infections (HSV-1 & HSV-2) trigger EM- minor
in almost 100% of cases

> Besides herpetic infection (55%), Mycoplasma Pneumoniae
appears to be common cause in EM Major and in Children.

» Some other viruses like CMV, VZV and HIV have been frequently
associated with EM

» Exposure to certain analgesics and antibiotics, particularly Sulfa-
drugs are most common triggers.

38. Clinical Features

> Occurs chiefly in young adults the highest incidence is in the

>
>

>

>

>

second to fourth decades of life.
M>F

Characterized by the occurrence of asymptomatic,
erythematous discrete macules, papules or occasionally vesicles
and bullae distributed in a rather symmetrical pattern most
commonly over the hands and arms, feet and legs, face and
neck.

A concentric ringlike appearance of the lesions, resulting from the
varying shades of erythema, ‘target’, ‘iris’, or ‘bull's eye”.

In oral cavity tongue, palate, buccal mucosa and gingiva are
commonly involved.

Occasionally, mucous membrane lesions occur before the
cutaneous manifestations

40. Steven-johnson Syndrome

> Cutaneous lesions — are similar to those of erythema
multiforme and they are commonly hemorrhagic

> Oral mucosa lesions = extremely severe, so painful that
mastication is impossible. Mucosal vesicles rupture and
leave surfaces covered with a thick white or yellow
exudate.

» Eye lesions — photophobia, comeal ulceration &
keratoconjuctivitis sicca finally leading to Blindness

> Genital lesions - nonspecpific urethritis, balanitis

» Other reported complications are related to
respiratory tract involvement.

41. Histological Features

> Epithelial hyperplasia, spongiosis.

> Subepithelial (skin lesions) / Intraepithelial

vesiculation (oral lesions) with necrotic basal
keratinocytes,

> Mixed inflammatory infiltrate arranged in
perivascular orientation.

43. Differential Diagnosis

Oral lesions:

Aphthous stomatitis

Contact dermatitis or Stomatitis andANUG
Skin lesions:

Pemphigus,

Dermatitis herpetiformis,

Bullous lichen planus

Hrpes zoster, and

Toxic epidermal necrolysis (Lyell's disease).