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General Pathology
Basic Principles of Cellular and Organ
Pathology
Autogenous
Pigments
Jaroslava Dušková
Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague
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PigmentsDefinition:
colored substances
in the organism
or environment
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PigmentsClassification:
� endogenous
� autogenous
� hemoproteins derived
� exogenous
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Autogenous Pigments
- color substances formed in the
organism as metabolism products
� melanin
� ceroid
� lipofuscin
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Melanin
� oculocutaneous
(origin from tyrosine in melanocytes)
� neuromelanin - subst. nigra
(origin from dopamin)
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Melanin - types
� eumelanin – insoluble , brown-black
� phaeomelanin – soluble, yellow-red
(high sulphur content)
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Melanin - production
Melanocytes
– derived from the neural crest
– present in the basal layer of epidermis,
dermis, hair folicles, mucose membranes,
uveal tract of the eye, meninges, inner ear
– secretory in the contact with the epithelial
cells - cytocrinia
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Melanin Functions – 1.
� cytoprotective
– light absorption & conversion of the photon
energy into heat
– uvea – absorption of the light retina
protection of light overexposure
– retina - visual acuity preventing light
reflexion from the fundus
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Melanin Functions -2.
� Ion exchanging capacity
Melanosomes can also act as detoxyfiing and
excretory components accumulating great number
of drugs and toxic component e.g. heavy metals.
Scavengers of the free radicals.
Rarely cytotoxic – photosensibilisation
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Melanin - Features
� brown
� destained with H2O2
� reducing AgNO3
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Disorders
of Melanin Pigmentation
Lack
� generalized
– total albinism
– parcial
albinism
� local
– vitiligo
– leucoderma
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Albinism
� autosomal recessive heredity
– tyrosinase deficiency
– tyrosinase positive – melanosomes defect
� oculo-cutaneous albinoidism – dominant
inheritance
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Disorders
of Melanin Pigmentation
Lack
� generalized
– total albinism
– parcial
albinism
� local
– vitiligo
– leucoderma
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Vitiligo
� familial aggregation
� polygenic nature
� association with other
autoimmune diseases (DM,
thyroiditis, gastritis)
� ab against tyrosinase in the serum
� autoreact . T- cellular cytotoxicity
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Disorders
of Melanin Pigmentation
Lack
� generalized
– total albinism
– parcial
albinism
� local
– vitiligo
– leucoderma
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Leucoderma
� postinflammatory
circumscribed depigmentation
e.g.
– leucoderma syphyliticum
– leucoderma psoriaticum
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Disorders
of Melanin Pigmentation
Increase
� generalized– Adison
disease
� local– freckles, nevi
– chloasma /melasma
–melanodermia
–melanoma
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Disorders
of Melanin Pigmentation
Increase
� generalized– Adison
disease
� local– freckles, nevi
– chloasma
–melanodermia
–melanoma
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Disorders of Phenylalanine
and Tyrosine Metabolism
1. Phenylalanine hydroxylase
PHENYLKETONURIA
2. Homogentisic acid oxydase
ALKAPTONURIA
3. Tyrosinase ALBINISM
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Disorders of Phenylalanine
and Tyrosine Metabolism
Phenylalanine Tyrosine
methyl–
acetoacetic
acid
DOPA
MELANINNorepinephrine
Epinephrine
3
1
Homogentisic
acid
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Ceroid� features
– light brown
– PAS +
– acidoresistent
– Sudan +-
� origin
– fagocytosis od lipid substances by macrophages
– oxidation of non–saturated lipid acids
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Ceroid
� localisation
– places of erythrocytes destruction
– necroses of adipous tissue
– avitamonosis E
– melanosis coli
– Dubin - Johnson syndrome
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Lipochrom
� ubiquitous pigment
� exogenous origin
� lipid solvent
� histologically unprovable
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Lipofuscin
� features – dark brown
– Sudan +-
– autofluorescence
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Lipofuscin
�origin – autophagocytosis
"wear and tear" pigment
from the accumulation
of autophagolysosomes over time.
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Lipofuscin
�localisation
– CNS, epithels, muscles, liver
ATROPHIA FUSCA