This document summarizes several developmental disturbances of the lip, palate, and oral mucosa. It describes congenital lip and commissural pits/fistulas, which can occur alone or with clefts. It also discusses Van der Woude syndrome, cleft lip and palate, cheilitis glandularis, cheilitis granulomatosa, hereditary intestinal polyposis syndrome, labial and oral melanotic macules, Fordyce's granules, and focal epithelial hyperplasia. For each condition, it provides information on etiology, clinical features, histological features if applicable, differential diagnosis, and treatment approaches.
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Developmental disturbances of LIP,PALATE and ORAL MUCOSA
3. Congenital lip and
commissural pits and fistula
It is malformation of the lips ,often following
a hereditary pattern.
It may occur alone or in association with other
developmental anomalies such as various oral
clefts.
75-80% of all cases of congenital labial fistulas
,there is an associated cleft lip or cleft palate.
4. ETIOLOGY:
Many theories have put up but none has been
universally accepted.
Notching of the lip.
Fixation of the tissue at the base of the notch.
C/F:
6. VAN DER WOUDE SYNDROME
It is an autosomal dominant syndrome
typically consisting of cleft lip or cleft palate
and distinctive pits of the lower lip
ETIOLOGY:
The most prominent feature is orofacial
anomalies
Caused due to abnormal fusion of palate
and lip , at days 30-50 postconception
7. C/F:
Occurences: affects about 1 in 100,000-
200,000.
Sex: no sex prediliction
Lesion: isolated ,usually medial
Site : on the vermilion portion of lower lip
8. TREATMENT:
Examination and genetic counseling by a
pediatric geneticist.
Surgical repair of clept lip and palate
9. CLEFT LIP AND CLEFT PALATE
It is a common congenital malformation.
Failure in the fusion of the nasal and
maxillary prosses leads to cleft of
primary palate ,can be unilateral or
bilateral.
Incidence of cleft of the lip and palate
varies from 1 in 500 to 1 in 2500
depends on geographic origin.
10. ETIOLOGY:
Heredity.
Environmental factors
Insufficent nutrition to pregnant women
OTHER FACTORS:
o Defective vascular supply
o Size of the tongue prevent union of affected
parts
o Infections , certain alcohol ,drugs and toxins
o Lack of inherent developmental force
11. C/F:
Sex : male predilection
Lesion: unilateral or bilateral anomaly
Types :
i. The cleft anterior to the incisive foramen is
defined as cleft of primary palate.
ii. The cleft posterior to the incisive foramen is
defined as a cleft of secondary palate.
12. CLINICAL SIGNIFICANCE:
Most cases can be surgically repaired
with excellent cosmetic and functional
results.
Eating and drinking are difficult because
of regurgitation of food and liquid
through the nose.
TREATMENT:
Surgical treatment
17. CHELITIS GLANDULARIS
Characterized by progressive enlargement
and eversion of the lower labial mucosa that
results in obliteration of the mucosal-
vermillion interface.
18. Etiology :
Chronic irritation.
Lip enlargement is attributable to
inflammation , hyperemia , edema and
fibrosis.
Surface keratosis , erosion,self-inflicted
biting , factitial trauma , excessive wetting
from compulsive licking , drying
Chronic aggravating factor.
19. C/F:
Lesion :enlargement of lip and loss of
elasticty , asymptomatic lip swelling ,
burning discomfort, sensation of rawness
Sex :male predilection.
Age :4th -6th decade.
Secretion: Mucopurulent exudates from
ductal orifices of labial minor salivary
glands.
22. CHEILITIS GRANULOMATOUS
Cheilitis granulomatosa is a chronic swelling
of the lip due to granulomatous
inflammation.
Etiology:
Cause is unknown.
23. C/F:
Non-tender swelling and enlargement one
or more lips and cheeks.
Enlarged lip appears cracked.
Fissured with reddish brown discoloration
and scaling.
25. Histological feature:
o Chronic inflammatory cell infiltrate
o Shows peri and para vascular aggregations
of lymphocytes ,plasma cells and
histiocytes.
o Formation with epitheloid cells and
Langhans type giant cells.
26. Treatment :
Intra lesional corticosteriods injections.
Non steroidal anti-inflammatory agents .
Mast cell stabilizers .
Clofazimine.
Tetracycline
Surgery and radiation.
28. Hereditory intestinal polyposis
syndrome
It is an autosomal dominantly inheritant disorder
characterized by intestinal hamaratomaous polyps
in association with muco-cutaneous melanocytic
macules.
Etiology:
The cause of the Peutz-Jeghers syndrome
appears to be a germline mutation of the
STK11 gene in most cases, located on band
19p13.3
29. Clinical feature:
>Sex: M=F
>Races: all races
>Signs and symptoms: intestinal bleeding, menstrual
irregularities, cutaneous pigmentation
Histological features:
>Extensive smooth muscle arborization throughout
the polyp.
Treatment:
>surgical treatment
32. Labial and oral melanotic
macule
It shows a focal area of melanin deposition
C/F:
Sex:2:1 female predilection
Age :23years
Site: vermilion border of lip , buccal mucosa,
gingiva and palate
Lesion: well demarcated, uniformly tan to
dark brown, asymptomatic ,round shaped
33. Histological feature:
o Normal stratified squamous epithelium with
abundant melanin deposits within the
keratinicytes of basal and parabasal layers.
TREATMENT:
No treatment is
required
36. FORDYCES GRANULES
It is a developmental anomaly characterized by
heterotropic collections of sebaceous glands at
various sites in the oral cavity
C/F:
Lesion :Small yellow spots.
Sex/race: No gender and races predilection.
Site: Tongue ,gingiva , frenum and palate
37. Histological feature:
o Heterotopic collections of sebaceous
gland.
o The gland are usually superficial and may
consist of only a few or a great many
lobules
o Shows keratin plugging.
Treatment:
Requires no treatment.
39. FOCAL EPITHELIAL HYPERPLASIA
It is one of the most contagious of the oral
papillary lesion.
It is able to produce extreme acantosis or
hyperplasia of the prickle cell layer of
epithelium.
40. C/F:
Age :children ,young and middle-aged.
Sex : no predilection
Site: labial , buccal and lingual mucosa ,
gingival.
Lesion: papillary in nature , smooth
surfaced, flat-topped ,pale or rarely white.
41. Histological features:
o Focal acantosis of oral epithelium
o Spinous layer show both cytoplasm and
nuclei in cell
Treatment:
Treatment is unnecessary.
Conservative excisional biopsy for proper
diagnosis.