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DISEASES
OF
THE SKIN
KERATOSIS FOLLICULARIS,
DYSKERATOSIS CONGENITA,
WHITE SPONGE NEVUS
INDIAN DENTAL ACADEMY
LEADER IN CONTINUING DENTAL EDUCATION
www.indiandentalacademy.com
LEARNING OBJECTIVES
At the end of the lecture student should be
describe
 Clinical features, Oral manifestations,&
Histopathological features Acanthosis Nigricans,
Ehlers-danlos syndrome, Focal dermal hypoplasia
syndrome (Goltzs syndrome).
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ACANTHOSIS NIGRICANS
Acanthosis nigricans is a rare cutaneous
disease, which usually affects the flexural
surfaces of skin and it has an oral
mucosal component.
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3 TYPES:
1.Benign type-
 Present at birth or during puberty.
 Inherited as autosomal dominant trait
 Never associated with internal malignancy.
2.Malignant type-
 Usually develops after the age of 40 years
 Invariably associated with internal
malignancies like adenocarcinomas or
lymphomas of the GI tract.
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3.Pseudoacanthosis type-
 Most common form of acanthosis nigrican
 Lesions develop around body creases as a
result of obesity.
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CLINICAL FEATURES
 Pigmented, papillary or verrucous growths over
the axilla, palms, soles & face, etc.
 Dorsum of the tongue exhibits hypertrophy of
the filiform papilla with development of a shaggy
appearance.
 Tongue also presents some areas of
papillomatous growth.
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 Lip is grossely enlarged (mainly the upper lip) and
its surface is dotted with small papillomatous
nodules especially at the commissure.
 Givgiva also exhibits hyperplastic changes and the
lesions are clinically similar to that of the
fibromatosis gingivae.
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 Buccal mucosa and palate show a velvety
white appearance with papillary projections
in some areas.
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HISTOPATHOLOGY
 Thickening of the epithelium with marked
acanthosis.
 Hyperkeratinization of the surface epithelium.
 Malignant form of the disease shows marked
epithelial hyperplasia and acanthosis.
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EHLERS-DANLOS
SYNDROME
•A group of hereditary disorders characterized
by defective collagen synthesis in various body
organs.
• Hyper mobility of the joints & increased laxity
of the skin, - "Rubber man"
•Excessive bruising tendency & defective
wound healing due to increased fragility of the
skin & blood vessels.
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ORAL MANIFESTATIONS
 Increased fragility of the oral mucosa.
Retarded wound healing
 Bleeding from the gingiva & oral mucosa
,Mobility of teeth.
 Enamel hypoplasia
 Loss of normal scalloping of the dentino-
enamel junctions of the tooth
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 Large pulp stones in the teeth &
formation of irregular type of dentin
 Hypermobility of the
temporomandibular joint.
 Difficulty in suturing the oral wounds.
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www.indiandentalacademy.com
Differential Diagnosis
 Hereditary benign intraepithelial dyskeratosis.
 Lichen planus.
 Hyperplastic candidiasis.
 Leukoplakia.
 Verrucous carcinoma.
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Histopathology
 White sponge nevus microscopically presents
mild to moderate hyper-parakeratosis of the
epithelium with acanthosis and intercellular
edema.
 There may be presence of some "vacuolated"
cells in the spinus cell layer having pyknotic
nuclei.
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FOCAL DERMAL HYPOPLASIA SYNDROME (GOLTZS
SYNDROME)
 Genetic disorder characterized by distinctive
skin abnormalities and variety of defects
affecting eyes , teeth, skeletal , urinary ,
gstrointestinal , cardiovascular and central
nervous system.
 The skin lesions appear to evoke as
accumulations of fat rather than hypoplasia
of the dermis.
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 Mnemoic FOCAL
 Female sex
 Osteopsthica striatia
 Coloboma
 Lobster claw deformity
 Absent ectodermis, mespdermis, neurodermis
elements.
 Affected individuals are recognized at birth or
prenatally.
 Also known as GOLTZ syndrome.
www.indiandentalacademy.com
Etiology:
 X-linked dominant inheritance pattren, common
findings of syndactyly, oligodactyly and
polydactyly.
 Clinical features:
 Characterized by relative focal absence of
dermis associated with herniation of
subcutaneous fat into the defects
 Skin atropy, streaky pigmentation &
telangiectasia
 Multiple papillomas of the mucosa.
www.indiandentalacademy.com
ORAL MANIFESTATIONS
 Papillomas of the lips is a striking feature.
 Teeth are commonly defective in size, shape
or structure
 Microdontia is a common finding.
 Cleft lip or palate may also be present.
www.indiandentalacademy.com
HISTOLOGIC FEATURES:
 Attenuation of dermal collagen fibres with
partial to complete absence of significant
portions of dermis,
 Change in appearance of adipose in the
cells in the dermis.
 If the accumulation of adipocytes is
pronounced it may cause the apparent
herniation of subcutaneous through the
thinned skin.
www.indiandentalacademy.com
SUMMARY
 Introduction, Clinical features, Oral
manifestations,& Histopathological features of
 Acanthosis Nigricans
 Ehlers-danlos syndrome
 Focal dermal hypoplasia syndrome (Goltzs
syndrome)
www.indiandentalacademy.com
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
www.indiandentalacademy.com
www.indiandentalacademy.com

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Diseases of skin 8/cosmetic dentistry courses

  • 1. DISEASES OF THE SKIN KERATOSIS FOLLICULARIS, DYSKERATOSIS CONGENITA, WHITE SPONGE NEVUS INDIAN DENTAL ACADEMY LEADER IN CONTINUING DENTAL EDUCATION www.indiandentalacademy.com
  • 2. LEARNING OBJECTIVES At the end of the lecture student should be describe  Clinical features, Oral manifestations,& Histopathological features Acanthosis Nigricans, Ehlers-danlos syndrome, Focal dermal hypoplasia syndrome (Goltzs syndrome). www.indiandentalacademy.com
  • 3. ACANTHOSIS NIGRICANS Acanthosis nigricans is a rare cutaneous disease, which usually affects the flexural surfaces of skin and it has an oral mucosal component. www.indiandentalacademy.com
  • 4. 3 TYPES: 1.Benign type-  Present at birth or during puberty.  Inherited as autosomal dominant trait  Never associated with internal malignancy. 2.Malignant type-  Usually develops after the age of 40 years  Invariably associated with internal malignancies like adenocarcinomas or lymphomas of the GI tract. www.indiandentalacademy.com
  • 5. 3.Pseudoacanthosis type-  Most common form of acanthosis nigrican  Lesions develop around body creases as a result of obesity. www.indiandentalacademy.com
  • 6. CLINICAL FEATURES  Pigmented, papillary or verrucous growths over the axilla, palms, soles & face, etc.  Dorsum of the tongue exhibits hypertrophy of the filiform papilla with development of a shaggy appearance.  Tongue also presents some areas of papillomatous growth. www.indiandentalacademy.com
  • 7.  Lip is grossely enlarged (mainly the upper lip) and its surface is dotted with small papillomatous nodules especially at the commissure.  Givgiva also exhibits hyperplastic changes and the lesions are clinically similar to that of the fibromatosis gingivae. www.indiandentalacademy.com
  • 8.  Buccal mucosa and palate show a velvety white appearance with papillary projections in some areas. www.indiandentalacademy.com
  • 9. HISTOPATHOLOGY  Thickening of the epithelium with marked acanthosis.  Hyperkeratinization of the surface epithelium.  Malignant form of the disease shows marked epithelial hyperplasia and acanthosis. www.indiandentalacademy.com
  • 10. EHLERS-DANLOS SYNDROME •A group of hereditary disorders characterized by defective collagen synthesis in various body organs. • Hyper mobility of the joints & increased laxity of the skin, - "Rubber man" •Excessive bruising tendency & defective wound healing due to increased fragility of the skin & blood vessels. www.indiandentalacademy.com
  • 11. ORAL MANIFESTATIONS  Increased fragility of the oral mucosa. Retarded wound healing  Bleeding from the gingiva & oral mucosa ,Mobility of teeth.  Enamel hypoplasia  Loss of normal scalloping of the dentino- enamel junctions of the tooth www.indiandentalacademy.com
  • 12.  Large pulp stones in the teeth & formation of irregular type of dentin  Hypermobility of the temporomandibular joint.  Difficulty in suturing the oral wounds. www.indiandentalacademy.com
  • 14. Differential Diagnosis  Hereditary benign intraepithelial dyskeratosis.  Lichen planus.  Hyperplastic candidiasis.  Leukoplakia.  Verrucous carcinoma. www.indiandentalacademy.com
  • 15. Histopathology  White sponge nevus microscopically presents mild to moderate hyper-parakeratosis of the epithelium with acanthosis and intercellular edema.  There may be presence of some "vacuolated" cells in the spinus cell layer having pyknotic nuclei. www.indiandentalacademy.com
  • 16. FOCAL DERMAL HYPOPLASIA SYNDROME (GOLTZS SYNDROME)  Genetic disorder characterized by distinctive skin abnormalities and variety of defects affecting eyes , teeth, skeletal , urinary , gstrointestinal , cardiovascular and central nervous system.  The skin lesions appear to evoke as accumulations of fat rather than hypoplasia of the dermis. www.indiandentalacademy.com
  • 17.  Mnemoic FOCAL  Female sex  Osteopsthica striatia  Coloboma  Lobster claw deformity  Absent ectodermis, mespdermis, neurodermis elements.  Affected individuals are recognized at birth or prenatally.  Also known as GOLTZ syndrome. www.indiandentalacademy.com
  • 18. Etiology:  X-linked dominant inheritance pattren, common findings of syndactyly, oligodactyly and polydactyly.  Clinical features:  Characterized by relative focal absence of dermis associated with herniation of subcutaneous fat into the defects  Skin atropy, streaky pigmentation & telangiectasia  Multiple papillomas of the mucosa. www.indiandentalacademy.com
  • 19. ORAL MANIFESTATIONS  Papillomas of the lips is a striking feature.  Teeth are commonly defective in size, shape or structure  Microdontia is a common finding.  Cleft lip or palate may also be present. www.indiandentalacademy.com
  • 20. HISTOLOGIC FEATURES:  Attenuation of dermal collagen fibres with partial to complete absence of significant portions of dermis,  Change in appearance of adipose in the cells in the dermis.  If the accumulation of adipocytes is pronounced it may cause the apparent herniation of subcutaneous through the thinned skin. www.indiandentalacademy.com
  • 21. SUMMARY  Introduction, Clinical features, Oral manifestations,& Histopathological features of  Acanthosis Nigricans  Ehlers-danlos syndrome  Focal dermal hypoplasia syndrome (Goltzs syndrome) www.indiandentalacademy.com
  • 22. 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 www.indiandentalacademy.com