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Fat’heya Zahran
Fat’heya Zahran
Professor, Cairo University
Professor, Cairo University
 Melanotic
 Melanotic

Localized

Diffuse

 Non Melanotic
 Non Melanotic
Localized:
Localized:

Ectodermal alteration
Ectodermal alteration

Acanthosis Nigericans
Acanthosis Nigericans

Increased melanin production:
Increased melanin production:

Café au lait spots e.g. polyostotic fibrous dysplasia
Café au lait spots e.g. polyostotic fibrous dysplasia
and multiple neurofibromatosis..
and multiple neurofibromatosis
Smoker’s melanosis
Smoker’s melanosis
Lichen planus
Lichen planus
Local irritation.
Local irritation.

Melanocyte proliferation:
Melanocyte proliferation:

Naevi
Naevi
Melanoma/malignant melanoma
Melanoma/malignant melanoma
Lentigo e.g. peutz Jegher’s syndrome
Lentigo e.g. peutz Jegher’s syndrome

Drug-Induced.
Drug-Induced.
Diffuse:
Diffuse:

Racial pigmentation (basilar melanosis)
Racial pigmentation (basilar melanosis)
Metabolic pigmentation
Metabolic pigmentation
Hemochromatosis
Hemochromatosis
Porphyria
Porphyria
Pellagra
Pellagra

Endocrine
Endocrine

Addison’s disease
Addison’s disease
Hyperpituitrism
Hyperpituitrism

Autoimmune
Autoimmune
HIV melanosis
HIV melanosis
Lung/pancreas malignancy
Lung/pancreas malignancy
Drug-induced
Drug-induced

Drugs causing melanin formation e.g. ACTH, busulfan
Drugs causing melanin formation e.g. ACTH, busulfan
Drugs forming complex with melanin e.g. minocyclin
Drugs forming complex with melanin e.g. minocyclin
Drug deposited in skin and mucous membrane e.g. silver & gold
Drug deposited in skin and mucous membrane e.g. silver & gold
Non Melanotic
Vascular lesions:
Vascular lesions:

Lipopigments:
Lipopigments:

Hemangiomas
Hemangiomas
Kaposi’s sarcoma
Kaposi’s sarcoma
Varices
Varices
Hereditary hemorrhagic
Hereditary hemorrhagic
telangiectasia
telangiectasia

Lipochrome
Lipochrome
(carotene)
(carotene)
Lipofuscin
Lipofuscin
Fordyce’s
Fordyce’s
granules
granules

RBCS:
RBCS:

Extravasation
Extravasation
Qualitative changes in Hb
Qualitative changes in Hb
Pigments resulting from
Pigments resulting from
RBC destruction
RBC destruction

Chromogenic
Chromogenic
bacteria
bacteria

Black hairy
Black hairy
tongue
tongue

Heavy metals
Heavy metals
intoxication
intoxication
Acanthosis Nigericans
Unusual dermatosis characterized by skin
Unusual dermatosis characterized by skin
hyperkeratosis and melanin pigmentation and
hyperkeratosis and melanin pigmentation and
white oral lesion.
white oral lesion.
Types:
Types:
Benign form
Benign form

Associated with insulin resistant diabetes (defect in
Associated with insulin resistant diabetes (defect in
insulin receptors).
insulin receptors).
Insulin will bind to receptor for ILGF → growth of the
Insulin will bind to receptor for ILGF → growth of the
cells instead of glucose utilization → hyperkeratosis,
cells instead of glucose utilization → hyperkeratosis,
acanthosis and papillomatosis.
acanthosis and papillomatosis.

Malignant form
Malignant form

Internal malignancy releases certain peptides→ stimulate
Internal malignancy releases certain peptides→ stimulate
melanin production.
melanin production.
Acanthosis Nigericans
Acanthosis Nigericans
Café au lait pigmentation
These lesions have the colour of coffee and cream that
varies from small to large diffuse macule.
They are found in two rare conditions;
neurofibromatosis and polyostotic fibrous dysplasia.

(A) Multiple neurofibromatosis (Von Recklinghausen’s
disease of skin)
It is an inherited autosomal dominant condition
which is characterized by proliferation of fibrous
element of nerve sheath and cafe au lait
Clinical Features
(1) Multiple nodules:
Sessile or pedunculated, superficial or deep
nodules that mainly affect the trunk.
Malignant transformation to neurogenic
sarcoma is common in 5 % of cases. The
lesion may involve the whole body causing
cosmetic problem.
Tongue involvement results in multiple
nodules due to proliferation of fibrous
element of lingual nerve. In addition,
macroglossia, scrotal tongue and enlarged
fungiform papillae had been reported
Multiple
Neurofibromatosis
Sessile Nodules with Café au
Lait Pigmentation
(2) Neurologic manifestation:
Centrally located lesions within bone cavities are
associated with neurological manifestations such as
pain, deafness, seizures, mental retardation and
headache.
Mandibular nerve involvement can be detected
radiographically as radiolucent area and is
associated with lip numbness and pain.

(3) Café au lait pigmentation.
(3) Café au lait pigmentation.
B) Polyostotic Fibrous Dysplasia(
Jaffe type
Jaffe type
Unilateral bone involvement
Unilateral bone involvement
Most bones are intact
Most bones are intact
Skin café au lait spots
Skin café au lait spots
No endocrinal disturbance
No endocrinal disturbance

Albright syndrome
Albright syndrome

Unilateral
Unilateral
Most bones are affected
Most bones are affected
Café au lait spots
Café au lait spots
Endocrinal disturbance is present:
Endocrinal disturbance is present:
present
Parathyroid
Parathyroid
Thyroid
Thyroid
Pituitary
Pituitary
Gonads (precocious puberty)
Gonads (precocious puberty)
Albright Syndrome
Oral Findings:
Slowly progressive expansion of jaw bone:
Jaw bone is replaced by fibrous tissue and the
cortex becomes thin leading to pathologic
fracture

Radiographically the lesion may show one of
these patterns:
Ground glass radio-opaque pattern
Mottled radiolucent & radio-opaque pattern
Unilocular or multilocular radiolucencv
Smoker’s Melanosis
It is basilar melanosis that occurs in some
It is basilar melanosis that occurs in some
smokers where tobacco smoke products
smokers where tobacco smoke products
stimulate synthesis of melanin.
stimulate synthesis of melanin.
It appears as diffuse brown flat macules,
It appears as diffuse brown flat macules,
mainly on anterior labial gingiva in
mainly on anterior labial gingiva in
cigarette smokers and on palate in pipe
cigarette smokers and on palate in pipe
smokers but may also appear on cheek and
smokers but may also appear on cheek and
lip mucosa.
lip mucosa.
Smoker’s Melanosis
Pigmented Lichen Planus
Lichen planus is dermatologic disease
Lichen planus is dermatologic disease
which is characterized by white papules,
which is characterized by white papules,
erosive and atrophic areas. Rarely
erosive and atrophic areas. Rarely
erosive or papular form may be
erosive or papular form may be
associated with diffuse melanosis.
associated with diffuse melanosis.
This increase in melanogenesis may be
This increase in melanogenesis may be
stimulated by infiltrate of T-lymphocytes
stimulated by infiltrate of T-lymphocytes
Pigmented Lichen Planus
Definition:
Definition:
It is benign proliferation of melanocytes
It is benign proliferation of melanocytes
that appears after birth and during
that appears after birth and during
childhood, reaching certain size then
childhood, reaching certain size then
remains static.
remains static.
Clinical Features:
(i) Nevocellular nevi:
Junctional nevi:
Nevus cells maintain their location at the junction of the
epithelium and connective tissue.
They are flat, brown round and oval lesions.

Compound nevi:
Nevus cells proliferate down into the connective tissue.
They are dome-shaped, brown lesions.

Intradermal/intramucosal nevi:
Nevus cells are localized in connective tissue.
They appear as elevated brown nodules.

(ii) Blue nevi:

The nevus cells reside deeply in connective tissue. On skin they
appear blue in colour because the overlying vessels dampen the
brown colour of melanin.
Raised painless sessile or pedunculated.
Raised painless sessile or pedunculated.
If melanomas become painful, with increased
If melanomas become painful, with increased
pigmentation, bleeding or ulceration this may
pigmentation, bleeding or ulceration this may
be a sign of malignancy.
be a sign of malignancy.
Malignant melanoma is a rare neoplasma of
Malignant melanoma is a rare neoplasma of
the oral cavity.
the oral cavity.
Most commonly found on the palate and
Most commonly found on the palate and
anterior labial gingiva.
anterior labial gingiva.
Peutz-Jegher’s Syndrome
Definition:
It is an autosomal dominant syndrome
that is characterized by multiple
intestinal polyposis and melanotic
macules mainly on the face and oral
cavity
Clinical Features:
Clinical Features:
Brown Pigmentation:
Brown Pigmentation:

Multiple melanotic macules appearing as freckles,
Multiple melanotic macules appearing as freckles,
mainly perioral, perinasal and perioccular.
mainly perioral, perinasal and perioccular.
Melanotic macules are present intraoral, mainly on
Melanotic macules are present intraoral, mainly on
palate and lip.
palate and lip.

Intestinal polyposis:
Intestinal polyposis:

Polyposis of small intestine may result in abdominal
Polyposis of small intestine may result in abdominal
pain, hemorrhage or intestinal obstruction ..
pain, hemorrhage or intestinal obstruction
Malignant transformation can occur.
Malignant transformation can occur.
Intestinal polyps are better to be diagnosed by
Intestinal polyps are better to be diagnosed by
barium enema.
barium enema.
Peutz-Jegher’s Syndrome
Physiologic Pigmentation
)(Racial Pigmentation

It is basilar melanosis that evolves in
childhood, commonly in dark-skinned
individuals.
It appears as multiple, diffuse flat
brown macules, mainly on gingiva,
labial and cheek mucosa.
Racial Pigmentation
)Hemochromatosis (Bronze Diabetes
Primary ::
Primary

Disorder of inborn error of iron metabolism secondary to
Disorder of inborn error of iron metabolism secondary to
increased intestinal iron absorption.
increased intestinal iron absorption.

Secondary:
Secondary:

Due to increased iron intake or increased destruction of
Due to increased iron intake or increased destruction of
RBCs.
RBCs.

Excess iron is deposited in various tissue and
Excess iron is deposited in various tissue and
organs:
organs:

In the liver producing liver cirrhosis.
In the liver producing liver cirrhosis.
In the pancreas producing diabetes mellitus.
In the pancreas producing diabetes mellitus.
In the adrenal gland inducing Addison’s disease.
In the adrenal gland inducing Addison’s disease.
In the heart inducing heart failure.
In the heart inducing heart failure.
Porphyrias
Hereditary diseases caused by abnormalities in the pathway
Hereditary diseases caused by abnormalities in the pathway
of heme biosynthesis, resulting in over production of
of heme biosynthesis, resulting in over production of
porphyrins and porphyrin precursors.
porphyrins and porphyrin precursors.

Two types are characterized by skin pigmentation:
Two types are characterized by skin pigmentation:
Cutaneous hepatic porphyria “Porphyria cutenea tarda”
Cutaneous hepatic porphyria “Porphyria cutenea tarda”

Starts as erythema and progresses to vesicles that become confluent
Starts as erythema and progresses to vesicles that become confluent
to form bullae, that heal by scar with skin hyperpigmentation.
to form bullae, that heal by scar with skin hyperpigmentation.

Congenital erythropoietic porphyria:
Congenital erythropoietic porphyria:

Excessive formation of porphyrin in bone marrow leads to its
Excessive formation of porphyrin in bone marrow leads to its
deposition on the skin producing photosensitivity → vesicular and
deposition on the skin producing photosensitivity → vesicular and
bullous reaction on the light exposed skin.
bullous reaction on the light exposed skin.
Vesicles contain serous fluid with red fluorescence.
Vesicles contain serous fluid with red fluorescence.
Healing is by scars and red brown hyperpigmentation.
Healing is by scars and red brown hyperpigmentation.
Urine is red in colour.
Urine is red in colour.
Lavender teeth: due to incorporation of porphyrins in decidous and
Lavender teeth: due to incorporation of porphyrins in decidous and
permanent teeth
permanent teeth
Pellagra
It is niacin (Nicotinic acid) deficiency.
It is niacin (Nicotinic acid) deficiency.
It progresses through:
It progresses through:

Dermatitis with melanin pigmentation of the
Dermatitis with melanin pigmentation of the
exposed skin surfaces (hands, feet and face).
exposed skin surfaces (hands, feet and face).
Dementia (impaired memory)
Dementia (impaired memory)
Diarrhea,
Diarrhea,
Death (if untreated)
Death (if untreated)

Oral manifestations:
Oral manifestations:

Stomatitis, Glossitis, Acute necrotizing ulcerative
Stomatitis, Glossitis, Acute necrotizing ulcerative
gingivitis, Sialorrhea, Angular cheilosis, Herpes
gingivitis, Sialorrhea, Angular cheilosis, Herpes
labialis, Deminution of taste sensation.
labialis, Deminution of taste sensation.
Endocrinopathic Pigmentation
Bronzing of skin and patchy melanosis of
the oral mucous membrane are features of
adrenal cortex insufficiency (Addison’s
disease) or hyperfunction of pituitary
gland (due to increased ACTH).
The oversecretion of ACTH results in
melanosis since it is a hormone that have
stimulating properties on melanocytes.
HIV Oral Melanosis
HIV positive patients show
hyperpigmentation of the nails and mucous
membrane. The etiology is unknown but
may be due to the destruction of adrenal
cortex (Addison’s disease).
It appears as diffuse flat brown macules,
mainly on buccal and labial mucosa.
HIV Oral Melanosis
Lipopigments
Lipochrome: “Carotene”
Lipochrome: “Carotene”

Carotenes are yellow or red fat soluble pigments found in
Carotenes are yellow or red fat soluble pigments found in
carrots, tomatoes, sweet potatoes, green leafy plants, milk,
carrots, tomatoes, sweet potatoes, green leafy plants, milk,
body fat and egg yolk.
body fat and egg yolk.
Carotenes found in plants consist mainly of two molecules
Carotenes found in plants consist mainly of two molecules
of vitamin A, Joined by a double bond ..Orally ingested
of vitamin A, Joined by a double bond Orally ingested
carotenes are broken down by Carotenase enzyme into
carotenes are broken down by Carotenase enzyme into
vitamin A, the main storage of vitamin A is in the liver.
vitamin A, the main storage of vitamin A is in the liver.
Excessive intake of carotene results in carotenemia
Excessive intake of carotene results in carotenemia
The resultant yellow colour of skin is known as
The resultant yellow colour of skin is known as
((carotenodermia).
carotenodermia).
Carotene is water insoluble so can not reach the eye..
Carotene is water insoluble so can not reach the eye
Lipochrome Pigments
Sebaceous glands:
Yellowish white spots on skin or mucous
membrane occur due to ectopic collection
of sebaceous glands.
It may occur in:
Oral cavity → Fordyce’s granules
Nipple → Montogomry tubercles
Upper eye lid → Mebomian glands.

The lipopigment is the colouring matter of
the sebum and fatty tissues.
Fordyce’s Granules
Fordyce’s Granules
Pigmentation Related to RBCs
Extravasation:
Extravasation:
May be due to trauma, bleeding
May be due to trauma, bleeding
tendency and B.V. abnormality.
tendency and B.V. abnormality.
Extravasated RBCs are red in colour
Extravasated RBCs are red in colour
then turn brown due to degradation of
then turn brown due to degradation of
Hb into hemosidrin ((localized iron
Hb into hemosidrin localized iron
overload).
overload).
Petechiae
Qualitative Changes in Hb:
Qualitative Changes in Hb:
Reduced hemoglobin-reduced hemoglobin is
Reduced hemoglobin-reduced hemoglobin is
associated with cyanosis and bluish
associated with cyanosis and bluish
discoloration of the skin and oral mucosa,
discoloration of the skin and oral mucosa,
secondary to heart and lung disease due to
secondary to heart and lung disease due to
over extraction of oxygen from hemoglobin.
over extraction of oxygen from hemoglobin.
Carboxy hemoglobin- carboxy hemoglobin is
Carboxy hemoglobin- carboxy hemoglobin is
pink (cherry red) color, due to inhalation of
pink (cherry red) color, due to inhalation of
carbon monoxide.
carbon monoxide.
Pigmentation due to RBCs destruction:

Hb
Globin
Globin

Heme
Heme
Fe++
Fe++
Iron overload:
Localized
Generalized

2α +2β
2α +2β
chains
chains

Proto-porphyrin
Proto-porphyrin
Porphyria:

Hepatic
Erythropoietic

Thalassemia
Jaundice:

Hemolytic
Obstructive
Hepatocellular

Sickle cell
anemia
Jaundice
A common manifestation of liver disease due to an increase
A common manifestation of liver disease due to an increase
of the yellow pigment bilirubin in the blood & tissue fluid.
of the yellow pigment bilirubin in the blood & tissue fluid.
A yellow tint appears in the sclera and is followed by yellow
A yellow tint appears in the sclera and is followed by yellow
coloration of the skin and mucous membrane.
coloration of the skin and mucous membrane.
Etiology:
Etiology:
Hemolytic jaundice::Excessive production of bilirubin due
Hemolytic jaundice Excessive production of bilirubin due
to abnormally RBCs.
to abnormally RBCs.
Hepatocellular Jaundice::e.g. Infective hepatitis with wide
Hepatocellular Jaundice e.g. Infective hepatitis with wide
spread damage of the liver with consequent disorganization
spread damage of the liver with consequent disorganization
of its structure.
of its structure.
Extrahepatic (obstructive) Jaundice::Due to obstruction of
Extrahepatic (obstructive) Jaundice Due to obstruction of
the bile duct system by gall stones or by carcinoma of the
the bile duct system by gall stones or by carcinoma of the
head of pancreas.
head of pancreas.
Jaundice
Hemangioma
Unilateral portwine stain of face 
Sturge-Weber syndrome (heamangioma)
Varices (varicositis)
Kaposi Sarcoma
Kaposi Sarcoma
Multifocal smooth, red
raised neoplasm,
characterized by
proliferation of blood
vessels.
Site: head, neck, tip of
nose & intra-orally the
palate is commonly
involved in AIDS patient.
Exogenous Pigmentations
Exogenous pigmentations include:
Black hairy tongue
Tattoo: Amalgam, graphite tattoo.
Metallic intoxication: lead, mercury,
bismuth.
Black Hairy Tongue
Exogenous pigmentation
(1) Amalgam tattoo
Etiology
Amalgam tattoo is due to iatrogenic factors. For
example:
Dentist’s bur loaded with small fragment of amalgam
particles that accumulate during removal of amalgam may
accidentally introduce the metal flecks into the oral
mucosa.
Metal particles may fall unnoticed into extraction socket.

Clinical Features
Grey or black macule on gingiva, palate or buccal
mucosa.
Amalgam tattoo is not harmful so its removal is not
required.
Biopsy is necessary if the lesion arises at areas distant from
any restoration to exclude melanoma
Amalgam Tattoo
(2) Graphite tattoo
It is due to traumatic implantation of graphite
from lead pencil, commonly seen on palate.
The patient may not recall the injury since the
event usually occurs during grade school.

(3) Metallic intoxication
High levels of heavy metals or metal salts may
result in metallic intoxication.
The exposure of the body to heavy metals may be
either:
Occupational hazard: as workers in lead factories.
Therapeutic hazard: as certain drugs, that contain salts
of heavy metals (bismuth, gold, mercury.
Clinical Features
Clinical Features
Systemic symptoms of toxicity including:
Systemic symptoms of toxicity including:
Behavioral change.
Behavioral change.
Intestinal pain.
Intestinal pain.
Neurological disorder.
Neurological disorder.

Oral features
Oral features

Grey to black pigmentation that outlines the
Grey to black pigmentation that outlines the
gingiva like “eyeliner”. The heavy metal tends
gingiva like “eyeliner”. The heavy metal tends
to extravasate from vessels in foci of increased
to extravasate from vessels in foci of increased
ability such as inflamed tissue. Thus the free
ability such as inflamed tissue. Thus the free
marginal gingiva is the most commonly affected
marginal gingiva is the most commonly affected
site.
site.
Mercurialism
Mercurialism
Lipochrome Pigments

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Pigmentation today

  • 1. By By Fat’heya Zahran Fat’heya Zahran Professor, Cairo University Professor, Cairo University
  • 2.  Melanotic  Melanotic Localized Diffuse  Non Melanotic  Non Melanotic
  • 3. Localized: Localized: Ectodermal alteration Ectodermal alteration Acanthosis Nigericans Acanthosis Nigericans Increased melanin production: Increased melanin production: Café au lait spots e.g. polyostotic fibrous dysplasia Café au lait spots e.g. polyostotic fibrous dysplasia and multiple neurofibromatosis.. and multiple neurofibromatosis Smoker’s melanosis Smoker’s melanosis Lichen planus Lichen planus Local irritation. Local irritation. Melanocyte proliferation: Melanocyte proliferation: Naevi Naevi Melanoma/malignant melanoma Melanoma/malignant melanoma Lentigo e.g. peutz Jegher’s syndrome Lentigo e.g. peutz Jegher’s syndrome Drug-Induced. Drug-Induced.
  • 4. Diffuse: Diffuse: Racial pigmentation (basilar melanosis) Racial pigmentation (basilar melanosis) Metabolic pigmentation Metabolic pigmentation Hemochromatosis Hemochromatosis Porphyria Porphyria Pellagra Pellagra Endocrine Endocrine Addison’s disease Addison’s disease Hyperpituitrism Hyperpituitrism Autoimmune Autoimmune HIV melanosis HIV melanosis Lung/pancreas malignancy Lung/pancreas malignancy Drug-induced Drug-induced Drugs causing melanin formation e.g. ACTH, busulfan Drugs causing melanin formation e.g. ACTH, busulfan Drugs forming complex with melanin e.g. minocyclin Drugs forming complex with melanin e.g. minocyclin Drug deposited in skin and mucous membrane e.g. silver & gold Drug deposited in skin and mucous membrane e.g. silver & gold
  • 5. Non Melanotic Vascular lesions: Vascular lesions: Lipopigments: Lipopigments: Hemangiomas Hemangiomas Kaposi’s sarcoma Kaposi’s sarcoma Varices Varices Hereditary hemorrhagic Hereditary hemorrhagic telangiectasia telangiectasia Lipochrome Lipochrome (carotene) (carotene) Lipofuscin Lipofuscin Fordyce’s Fordyce’s granules granules RBCS: RBCS: Extravasation Extravasation Qualitative changes in Hb Qualitative changes in Hb Pigments resulting from Pigments resulting from RBC destruction RBC destruction Chromogenic Chromogenic bacteria bacteria Black hairy Black hairy tongue tongue Heavy metals Heavy metals intoxication intoxication
  • 6. Acanthosis Nigericans Unusual dermatosis characterized by skin Unusual dermatosis characterized by skin hyperkeratosis and melanin pigmentation and hyperkeratosis and melanin pigmentation and white oral lesion. white oral lesion. Types: Types: Benign form Benign form Associated with insulin resistant diabetes (defect in Associated with insulin resistant diabetes (defect in insulin receptors). insulin receptors). Insulin will bind to receptor for ILGF → growth of the Insulin will bind to receptor for ILGF → growth of the cells instead of glucose utilization → hyperkeratosis, cells instead of glucose utilization → hyperkeratosis, acanthosis and papillomatosis. acanthosis and papillomatosis. Malignant form Malignant form Internal malignancy releases certain peptides→ stimulate Internal malignancy releases certain peptides→ stimulate melanin production. melanin production.
  • 9. Café au lait pigmentation These lesions have the colour of coffee and cream that varies from small to large diffuse macule. They are found in two rare conditions; neurofibromatosis and polyostotic fibrous dysplasia. (A) Multiple neurofibromatosis (Von Recklinghausen’s disease of skin) It is an inherited autosomal dominant condition which is characterized by proliferation of fibrous element of nerve sheath and cafe au lait
  • 10. Clinical Features (1) Multiple nodules: Sessile or pedunculated, superficial or deep nodules that mainly affect the trunk. Malignant transformation to neurogenic sarcoma is common in 5 % of cases. The lesion may involve the whole body causing cosmetic problem. Tongue involvement results in multiple nodules due to proliferation of fibrous element of lingual nerve. In addition, macroglossia, scrotal tongue and enlarged fungiform papillae had been reported
  • 12. Sessile Nodules with Café au Lait Pigmentation
  • 13. (2) Neurologic manifestation: Centrally located lesions within bone cavities are associated with neurological manifestations such as pain, deafness, seizures, mental retardation and headache. Mandibular nerve involvement can be detected radiographically as radiolucent area and is associated with lip numbness and pain. (3) Café au lait pigmentation.
  • 14. (3) Café au lait pigmentation.
  • 15. B) Polyostotic Fibrous Dysplasia( Jaffe type Jaffe type Unilateral bone involvement Unilateral bone involvement Most bones are intact Most bones are intact Skin café au lait spots Skin café au lait spots No endocrinal disturbance No endocrinal disturbance Albright syndrome Albright syndrome Unilateral Unilateral Most bones are affected Most bones are affected Café au lait spots Café au lait spots Endocrinal disturbance is present: Endocrinal disturbance is present: present Parathyroid Parathyroid Thyroid Thyroid Pituitary Pituitary Gonads (precocious puberty) Gonads (precocious puberty)
  • 17. Oral Findings: Slowly progressive expansion of jaw bone: Jaw bone is replaced by fibrous tissue and the cortex becomes thin leading to pathologic fracture Radiographically the lesion may show one of these patterns: Ground glass radio-opaque pattern Mottled radiolucent & radio-opaque pattern Unilocular or multilocular radiolucencv
  • 18. Smoker’s Melanosis It is basilar melanosis that occurs in some It is basilar melanosis that occurs in some smokers where tobacco smoke products smokers where tobacco smoke products stimulate synthesis of melanin. stimulate synthesis of melanin. It appears as diffuse brown flat macules, It appears as diffuse brown flat macules, mainly on anterior labial gingiva in mainly on anterior labial gingiva in cigarette smokers and on palate in pipe cigarette smokers and on palate in pipe smokers but may also appear on cheek and smokers but may also appear on cheek and lip mucosa. lip mucosa.
  • 20. Pigmented Lichen Planus Lichen planus is dermatologic disease Lichen planus is dermatologic disease which is characterized by white papules, which is characterized by white papules, erosive and atrophic areas. Rarely erosive and atrophic areas. Rarely erosive or papular form may be erosive or papular form may be associated with diffuse melanosis. associated with diffuse melanosis. This increase in melanogenesis may be This increase in melanogenesis may be stimulated by infiltrate of T-lymphocytes stimulated by infiltrate of T-lymphocytes
  • 22. Definition: Definition: It is benign proliferation of melanocytes It is benign proliferation of melanocytes that appears after birth and during that appears after birth and during childhood, reaching certain size then childhood, reaching certain size then remains static. remains static.
  • 23. Clinical Features: (i) Nevocellular nevi: Junctional nevi: Nevus cells maintain their location at the junction of the epithelium and connective tissue. They are flat, brown round and oval lesions. Compound nevi: Nevus cells proliferate down into the connective tissue. They are dome-shaped, brown lesions. Intradermal/intramucosal nevi: Nevus cells are localized in connective tissue. They appear as elevated brown nodules. (ii) Blue nevi: The nevus cells reside deeply in connective tissue. On skin they appear blue in colour because the overlying vessels dampen the brown colour of melanin.
  • 24.
  • 25. Raised painless sessile or pedunculated. Raised painless sessile or pedunculated. If melanomas become painful, with increased If melanomas become painful, with increased pigmentation, bleeding or ulceration this may pigmentation, bleeding or ulceration this may be a sign of malignancy. be a sign of malignancy. Malignant melanoma is a rare neoplasma of Malignant melanoma is a rare neoplasma of the oral cavity. the oral cavity. Most commonly found on the palate and Most commonly found on the palate and anterior labial gingiva. anterior labial gingiva.
  • 26.
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  • 29. Peutz-Jegher’s Syndrome Definition: It is an autosomal dominant syndrome that is characterized by multiple intestinal polyposis and melanotic macules mainly on the face and oral cavity
  • 30. Clinical Features: Clinical Features: Brown Pigmentation: Brown Pigmentation: Multiple melanotic macules appearing as freckles, Multiple melanotic macules appearing as freckles, mainly perioral, perinasal and perioccular. mainly perioral, perinasal and perioccular. Melanotic macules are present intraoral, mainly on Melanotic macules are present intraoral, mainly on palate and lip. palate and lip. Intestinal polyposis: Intestinal polyposis: Polyposis of small intestine may result in abdominal Polyposis of small intestine may result in abdominal pain, hemorrhage or intestinal obstruction .. pain, hemorrhage or intestinal obstruction Malignant transformation can occur. Malignant transformation can occur. Intestinal polyps are better to be diagnosed by Intestinal polyps are better to be diagnosed by barium enema. barium enema.
  • 32.
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  • 36. Physiologic Pigmentation )(Racial Pigmentation It is basilar melanosis that evolves in childhood, commonly in dark-skinned individuals. It appears as multiple, diffuse flat brown macules, mainly on gingiva, labial and cheek mucosa.
  • 38. )Hemochromatosis (Bronze Diabetes Primary :: Primary Disorder of inborn error of iron metabolism secondary to Disorder of inborn error of iron metabolism secondary to increased intestinal iron absorption. increased intestinal iron absorption. Secondary: Secondary: Due to increased iron intake or increased destruction of Due to increased iron intake or increased destruction of RBCs. RBCs. Excess iron is deposited in various tissue and Excess iron is deposited in various tissue and organs: organs: In the liver producing liver cirrhosis. In the liver producing liver cirrhosis. In the pancreas producing diabetes mellitus. In the pancreas producing diabetes mellitus. In the adrenal gland inducing Addison’s disease. In the adrenal gland inducing Addison’s disease. In the heart inducing heart failure. In the heart inducing heart failure.
  • 39. Porphyrias Hereditary diseases caused by abnormalities in the pathway Hereditary diseases caused by abnormalities in the pathway of heme biosynthesis, resulting in over production of of heme biosynthesis, resulting in over production of porphyrins and porphyrin precursors. porphyrins and porphyrin precursors. Two types are characterized by skin pigmentation: Two types are characterized by skin pigmentation: Cutaneous hepatic porphyria “Porphyria cutenea tarda” Cutaneous hepatic porphyria “Porphyria cutenea tarda” Starts as erythema and progresses to vesicles that become confluent Starts as erythema and progresses to vesicles that become confluent to form bullae, that heal by scar with skin hyperpigmentation. to form bullae, that heal by scar with skin hyperpigmentation. Congenital erythropoietic porphyria: Congenital erythropoietic porphyria: Excessive formation of porphyrin in bone marrow leads to its Excessive formation of porphyrin in bone marrow leads to its deposition on the skin producing photosensitivity → vesicular and deposition on the skin producing photosensitivity → vesicular and bullous reaction on the light exposed skin. bullous reaction on the light exposed skin. Vesicles contain serous fluid with red fluorescence. Vesicles contain serous fluid with red fluorescence. Healing is by scars and red brown hyperpigmentation. Healing is by scars and red brown hyperpigmentation. Urine is red in colour. Urine is red in colour. Lavender teeth: due to incorporation of porphyrins in decidous and Lavender teeth: due to incorporation of porphyrins in decidous and permanent teeth permanent teeth
  • 40.
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  • 45. Pellagra It is niacin (Nicotinic acid) deficiency. It is niacin (Nicotinic acid) deficiency. It progresses through: It progresses through: Dermatitis with melanin pigmentation of the Dermatitis with melanin pigmentation of the exposed skin surfaces (hands, feet and face). exposed skin surfaces (hands, feet and face). Dementia (impaired memory) Dementia (impaired memory) Diarrhea, Diarrhea, Death (if untreated) Death (if untreated) Oral manifestations: Oral manifestations: Stomatitis, Glossitis, Acute necrotizing ulcerative Stomatitis, Glossitis, Acute necrotizing ulcerative gingivitis, Sialorrhea, Angular cheilosis, Herpes gingivitis, Sialorrhea, Angular cheilosis, Herpes labialis, Deminution of taste sensation. labialis, Deminution of taste sensation.
  • 46.
  • 47.
  • 48. Endocrinopathic Pigmentation Bronzing of skin and patchy melanosis of the oral mucous membrane are features of adrenal cortex insufficiency (Addison’s disease) or hyperfunction of pituitary gland (due to increased ACTH). The oversecretion of ACTH results in melanosis since it is a hormone that have stimulating properties on melanocytes.
  • 49.
  • 50. HIV Oral Melanosis HIV positive patients show hyperpigmentation of the nails and mucous membrane. The etiology is unknown but may be due to the destruction of adrenal cortex (Addison’s disease). It appears as diffuse flat brown macules, mainly on buccal and labial mucosa.
  • 52. Lipopigments Lipochrome: “Carotene” Lipochrome: “Carotene” Carotenes are yellow or red fat soluble pigments found in Carotenes are yellow or red fat soluble pigments found in carrots, tomatoes, sweet potatoes, green leafy plants, milk, carrots, tomatoes, sweet potatoes, green leafy plants, milk, body fat and egg yolk. body fat and egg yolk. Carotenes found in plants consist mainly of two molecules Carotenes found in plants consist mainly of two molecules of vitamin A, Joined by a double bond ..Orally ingested of vitamin A, Joined by a double bond Orally ingested carotenes are broken down by Carotenase enzyme into carotenes are broken down by Carotenase enzyme into vitamin A, the main storage of vitamin A is in the liver. vitamin A, the main storage of vitamin A is in the liver. Excessive intake of carotene results in carotenemia Excessive intake of carotene results in carotenemia The resultant yellow colour of skin is known as The resultant yellow colour of skin is known as ((carotenodermia). carotenodermia). Carotene is water insoluble so can not reach the eye.. Carotene is water insoluble so can not reach the eye
  • 54. Sebaceous glands: Yellowish white spots on skin or mucous membrane occur due to ectopic collection of sebaceous glands. It may occur in: Oral cavity → Fordyce’s granules Nipple → Montogomry tubercles Upper eye lid → Mebomian glands. The lipopigment is the colouring matter of the sebum and fatty tissues.
  • 57. Pigmentation Related to RBCs Extravasation: Extravasation: May be due to trauma, bleeding May be due to trauma, bleeding tendency and B.V. abnormality. tendency and B.V. abnormality. Extravasated RBCs are red in colour Extravasated RBCs are red in colour then turn brown due to degradation of then turn brown due to degradation of Hb into hemosidrin ((localized iron Hb into hemosidrin localized iron overload). overload).
  • 58.
  • 60. Qualitative Changes in Hb: Qualitative Changes in Hb: Reduced hemoglobin-reduced hemoglobin is Reduced hemoglobin-reduced hemoglobin is associated with cyanosis and bluish associated with cyanosis and bluish discoloration of the skin and oral mucosa, discoloration of the skin and oral mucosa, secondary to heart and lung disease due to secondary to heart and lung disease due to over extraction of oxygen from hemoglobin. over extraction of oxygen from hemoglobin. Carboxy hemoglobin- carboxy hemoglobin is Carboxy hemoglobin- carboxy hemoglobin is pink (cherry red) color, due to inhalation of pink (cherry red) color, due to inhalation of carbon monoxide. carbon monoxide.
  • 61. Pigmentation due to RBCs destruction: Hb Globin Globin Heme Heme Fe++ Fe++ Iron overload: Localized Generalized 2α +2β 2α +2β chains chains Proto-porphyrin Proto-porphyrin Porphyria: Hepatic Erythropoietic Thalassemia Jaundice: Hemolytic Obstructive Hepatocellular Sickle cell anemia
  • 62. Jaundice A common manifestation of liver disease due to an increase A common manifestation of liver disease due to an increase of the yellow pigment bilirubin in the blood & tissue fluid. of the yellow pigment bilirubin in the blood & tissue fluid. A yellow tint appears in the sclera and is followed by yellow A yellow tint appears in the sclera and is followed by yellow coloration of the skin and mucous membrane. coloration of the skin and mucous membrane. Etiology: Etiology: Hemolytic jaundice::Excessive production of bilirubin due Hemolytic jaundice Excessive production of bilirubin due to abnormally RBCs. to abnormally RBCs. Hepatocellular Jaundice::e.g. Infective hepatitis with wide Hepatocellular Jaundice e.g. Infective hepatitis with wide spread damage of the liver with consequent disorganization spread damage of the liver with consequent disorganization of its structure. of its structure. Extrahepatic (obstructive) Jaundice::Due to obstruction of Extrahepatic (obstructive) Jaundice Due to obstruction of the bile duct system by gall stones or by carcinoma of the the bile duct system by gall stones or by carcinoma of the head of pancreas. head of pancreas.
  • 65. Unilateral portwine stain of face  Sturge-Weber syndrome (heamangioma)
  • 68. Kaposi Sarcoma Multifocal smooth, red raised neoplasm, characterized by proliferation of blood vessels. Site: head, neck, tip of nose & intra-orally the palate is commonly involved in AIDS patient.
  • 69. Exogenous Pigmentations Exogenous pigmentations include: Black hairy tongue Tattoo: Amalgam, graphite tattoo. Metallic intoxication: lead, mercury, bismuth.
  • 71. Exogenous pigmentation (1) Amalgam tattoo Etiology Amalgam tattoo is due to iatrogenic factors. For example: Dentist’s bur loaded with small fragment of amalgam particles that accumulate during removal of amalgam may accidentally introduce the metal flecks into the oral mucosa. Metal particles may fall unnoticed into extraction socket. Clinical Features Grey or black macule on gingiva, palate or buccal mucosa. Amalgam tattoo is not harmful so its removal is not required. Biopsy is necessary if the lesion arises at areas distant from any restoration to exclude melanoma
  • 73. (2) Graphite tattoo It is due to traumatic implantation of graphite from lead pencil, commonly seen on palate. The patient may not recall the injury since the event usually occurs during grade school. (3) Metallic intoxication High levels of heavy metals or metal salts may result in metallic intoxication. The exposure of the body to heavy metals may be either: Occupational hazard: as workers in lead factories. Therapeutic hazard: as certain drugs, that contain salts of heavy metals (bismuth, gold, mercury.
  • 74. Clinical Features Clinical Features Systemic symptoms of toxicity including: Systemic symptoms of toxicity including: Behavioral change. Behavioral change. Intestinal pain. Intestinal pain. Neurological disorder. Neurological disorder. Oral features Oral features Grey to black pigmentation that outlines the Grey to black pigmentation that outlines the gingiva like “eyeliner”. The heavy metal tends gingiva like “eyeliner”. The heavy metal tends to extravasate from vessels in foci of increased to extravasate from vessels in foci of increased ability such as inflamed tissue. Thus the free ability such as inflamed tissue. Thus the free marginal gingiva is the most commonly affected marginal gingiva is the most commonly affected site. site.
  • 75.