Oral lesions with dyskeratosis

Lesions associated with

abnormal dyskeratosisMade by :

Yousef Abosaif

Definition

Dyskeratosis is an Abnormal keratinization of epithelial

cells

types

1 – benign dyskeratosis

2 – Malignant dyskeratosis

1- Benign dyskeratosis

1- Hereditary Benign Interepithelial

dyskeratosis (HBID)

2- Dyskeratosis congenita

3- keratoacanthoma

4- Darrier’s disease

5- WARTY DYSKERATOMA

2 – Malignant dyskeratosis

• Found in malignant and premalignant conditions

e.g leukoplakia and bowen's disease

1- Dyskeratosis congenita

rare genodermatosis that is usually inherited as an X-

linked recessive trait.

Autosomal dominant and autosomal recessive forms,

although less common, have been reported.

The clinician should be aware of the condition

because the oral lesions may undergo malignant

transformation, and patients are susceptible to aplastic

anemia.

Clinical features

becomes evident during the first 10 years of

life.

Intraorally, the tongue and buccal mucosa

develop bullae; these are followed by erosions

and, eventually, leukoplakic lesions

one third of them become malignant in a 10- to

30- year period

Aplastic anemia develops in approximately

70% of these patients

Dyskeratosis congenita. Dysplastic nail

changes.

Dyskeratosis congenita. Atrophy and

hyperkeratosis of the dorsal tongue

mucosa are visible.

HISTOPATHOLOGIC FEATURES

Biopsy specimens of the early oral

mucosal lesions show

hyperorthokeratosis with epithelial

atrophy. As the lesions progress,

epithelial dysplasia develops until frank

squamous cell carcinoma evolves.

TREATMENT AND PROGNOSIS

The discomfort of the oral lesions is managed

symptomatically,and careful periodic oral mucosal

examinations are performed to check for evidence of

malignant transformation.

Routine medical evaluation is warranted to monitor the

patient for the development of aplastic anemia.

2-keratoacanthoma

• self-limiting, epithelial proliferation with a strong

clinical and histopathologic similarity to well-

differentiated squamous cell carcinoma

• The cause of this lesion is unknown, but sun

damage and human papillomavirus (HPV), possibly

subtypes 26 or 37, have been proposed.

CLINICAL FEATURES

• rarely occurs in patients before 45 years of age and

shows a male predilection.

• Keratoacanthoma appears as a firm, nontender,

well-demarcated, sessile, dome-shaped nodule with

a central plug of keratin

• Almost 95% of solitary lesions are found on sun-

exposed skin, and 8% of all cases are found on the

outer edge of the vermilion border of the lips, with

equal frequency on boththe upper and the lower lips

Keratoacanthoma. A nontender,

welldemarcated nodule of the skin of the

nose in an older woman. The nodule

demonstrates a central keratin plug.

Keratoacanthoma. This lesion, which

is located at the outer edge of the

vermilion border of the lip,

demonstrates a prominent core or plug

of keratin.


• cells appear mature, although considerable

dyskeratosis is typically seen in the form of deeply

located individually keratinizing lesional cells and

keratin pearls similar to those found in well-

differentiated squamous cell carcinoma.

Keratoacanthoma. Low-power

microscopic view showing

extensive epidermal proliferation

with a central keratin plug.


• surgical excision of large lesions is indicated for optimal aesthetic appearance because signifi cant scarring may otherwise occur.

• 4% to 8% of treated patients experience recurrence.

• alternative therapies include cryosurgery (reserved for small early lesions), intralesional injection of chemotherapeutic agents and topical imiquimod.

• Systemic chemotherapy, often combined with cryotherapy, may be used to treat patients with multiple lesions

3-HEREDITARY BENIGN

INTRAEPITHELIAL DYSKERATOSIS

• Hereditary benign intraepithelial dyskeratosis

(HBID) is a rare autosomal dominant

genodermatosis

Hereditary benign intraepithelial

dyskeratosis(HBID).

Oral lesions appear as corrugated

white plaques of the buccal

mucosa.

CLINICAL FEATURES

• usually develop during childhood,

• in most instances affecting the oral and conjunctival

mucosa.

• The oral lesions are similar to those of white sponge

nevus, with both conditions showing

thick, corrugated white plaques involving the buccal

and labial mucosa


• Prominent parakeratin production in addition to

marked acanthosis

• With this dyskeratotic process, an epithelial cell

appears to be surrounded or engulfed by an

adjacent epithelial cell, resulting in the so-called cell-

withina-cell phenomenon

A

b

Hereditary benign intraepithelial

dyskeratosis

(HBID). A, Medium-power

photomicrograph exhibiting

hyperparakeratosis, acanthosis, and

dyskeratosis. B, Higher

magnifi cation showing dyskeratotic cells.


• Because HBID is a benign condition, no treatment is

generally required or indicated for the oral lesions.

• If superimposed candidiasis develops, then an

antifungal medication can be used

• Patients with symptomatic ocular lesions should be

referred to an ophthalmologist.

4-DARIER’S DISEASE

• is inherited as an autosomal dominant trait, having a

high degree of penetrance and variable expressivity.

• A lack of cohesion among the surface epithelial cells

characterizes this disease, and mutation of a gene

that encodes an intracellular calcium pump has

been identified as the cause for abnormal

desmosomal organization in the affected epithelial

cells.

CLINICAL FEATURES

• Patients with Darier’s disease have numerous

erythematous papules on the skin of the trunk and

the scalp that develop during the first or second

decade of life

• The oral lesions are typically asymptomatic and are

discovered on routine examination. The frequency of

occurrence of oral lesions ranges from 15% to 50%.

• They consist of multiple, normal-colored or white, fl

attopped papules

Darier’s disease. Erythematous

cutaneous

papules on the chest.

Darier’s disease. The oral mucosa

may show multiple white papules


Darier’s disease. Low-power

photomicrograph

showing a thick keratin plug, intraepithelial

clefting, and elongated rete ridges.

a dyskeratotic process characterized by a central keratin

plug that overlies epithelium exhibiting a intraepithelial

cleft.


• Treatment of Darier’s disease depends on the

severity of involvement.

• Photosensitive patients should use a sunscreen,

and all patients should minimize unnecessary

exposure to hot environments.

• For relatively mild cases, keratolytic agents or

emollients may be the only treatment required. For

more severely affected patients,systemic retinoids

are often beneficial,

5-WARTY DYSKERATOMA

• is a distinctly uncommon solitary lesion that can

occur on skin or oral mucosa.

• It is histopathologically identical to Darier’s disease.

• For this reason the lesion has been termed isolated

Darier’s disease.

• The lesion is not otherwise related to Darier’s

disease, however, and its cause remains unknown.

CLINICAL FEATURES

• appears as a solitary, asymptomatic, umbilicated

papule on the skin of the head or neck of an older

adult

• intraoral warty dyskeratoma appears as a pink or

white, umbilicated papule located on the keratinized

mucosa,

especially the hard palate and the alveolar ridge

Warty dyskeratoma. Umbilicated papule

on

the hard palate.

Warty dyskeratoma. Well-circumscribed

invagination filled with a thick parakeratin

plug. There is hyperplasia of the basilar

cells with a suprabasilar cleft

Histopathologically, display dyskeratosis, basilar hyperplasia, and a

suprabasilar cleft


• Treatment of the warty dyskeratoma consists of

conservative excision.

• The prognosis is excellent; these lesions have not

been reported to recur, and they have no apparent

malignant potential.

LEUKOPLAKIA

• Is defined by the World Health Organization (WHO) as

“a white patch or plaque that cannot be characterized

clinically or pathologically as any other disease.”

• the clinicalcolor results from a thickened surface

keratin layer

• it is typically consideredto be a precancerous or

premalignant lesion

CAUSE

• The cause of leukoplakia remains unknown, although hypotheses abound.

• TOBACCO

• ALCOHOL

• SANGUINARIA

• ULTRAVIOLET RADIATION

• MICROORGANISMS

• TRAUMA

CLINICAL FEATURES

• usually affects persons older than 40 years of age

• Approximately 70% of oral leukoplakias are found

on the lip vermilion, buccal mucosa, and gingiva.

• Early and mild lesions appear as slightly elevated

gray or gray-white plaques.

• A special high-risk form of leukoplakia, proliferative

verrucous leukoplakia (PVL), is characterized by

the development of multiple keratotic plaques with

roughened surface projections

• Leukoplakia may become dysplastic, even invasive,

with no change in its clinical appearance.

Early or thin leukoplakia.

This early lesion of the

ventral tongue is smooth,

white, and well demarcated

from the surrounding normal

mucosa.

Homogeneous or thick

leukoplakia. A diffuse,

corrugated white patch on the

right ventral surface of the

tongue and fl oor of mouth.

Granular leukoplakia.

Irregular white patch in

the fl oor of the mouth of

a heavy smoker. Early

invasivesquamous cell

carcinoma was found on

biopsy.

Verruciform leukoplakia. Exophytic

papillary lesion of the anterior maxillary

alveolar ridge. Biopsy revealed

a well-differentiated squamous cell

carcinoma.

Proliferative verrucous leukoplakia (PVL). A, Large, diffuse, and

corrugated

white lesions of the buccal mucosa and tongue. B, Same patient showing

the extensive thickened and fi ssured alteration of the tongue.


• is characterized by a thickened keratin layer of the

surface epithelium (hyperkeratosis), with or without a

thickened spinous layer (acanthosis).

• The keratin layer may consist of parakeratin,

orthokeratin or a combination of both

• epithelial dysplasia is found in only 5% to 25% of

cases

The histopathologic alterations of dysplastic epithelial cells are similar to those of squamous cell carcinoma and may include the following:

• Enlarged nuclei and cells

• Large and prominent nucleoli

• Hyperchromatic (excessively dark-staining) nuclei

• Pleomorphic (abnormally shaped) nuclei and cells

• Dyskeratosis (premature keratinization of individual cells)

• Increased mitotic activity (excessive numbers of mitoses)

• Abnormal mitotic figures

Mild epithelial dysplasia.

Hyperchromatic and slightly pleomorphic

nuclei are noted in the basal and

parabasal cell layers of this stratifi ed

squamous epithelium.

Moderate epithelial dysplasia.

Dysplastic

changes extend to the midpoint of the

epithelium and are characterized by

nuclear hyperchromatism,

pleomorphism, and cellular crowding.

Severe epithelial dysplasia. Epithelium

exhibiting marked pleomorphism,

hyperchromatism, and scattered mitotic

figures. Atypical cells involve most of the

epithelial thickness.

TREATMENT

• Leukoplakia exhibiting moderate epithelial dysplasia

or worse warrants complete destruction or removal,

if feasible.

• The management of leukoplakia exhibiting less

severe change is guided by the size of the lesion

and the response to more conservative measures,

such as smoking cessation.

Thank You

Reference : Oral and Maxillofacial Pathology - Neville, Brad W.

Oral lesions with dyskeratosis

Health & Medicine