autogenous pigments -...
TRANSCRIPT
General Pathology
Basic Principles of Cellular and Organ
Pathology
Autogenous
Pigments
Jaroslava Dušková
Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague
PigmentsDefinition:
colored substances
in the organism
or environment
PigmentsClassification:
� endogenous
� autogenous
� hemoproteins derived
� exogenous
Autogenous Pigments
- color substances formed in the
organism as metabolism products
� melanin
� ceroid
� lipofuscin
Melanin
� oculocutaneous
(origin from tyrosine in melanocytes)
� neuromelanin - subst. nigra
(origin from dopamin)
Melanin - types
� eumelanin – insoluble , brown-black
� phaeomelanin – soluble, yellow-red
(high sulphur content)
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
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
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
Melanin - Features
� brown
� destained with H2O2
� reducing AgNO3
Disorders
of Melanin Pigmentation
Lack
� generalized
– total albinism
– parcial
albinism
� local
– vitiligo
– leucoderma
Albinism
� autosomal recessive heredity
– tyrosinase deficiency
– tyrosinase positive – melanosomes defect
� oculo-cutaneous albinoidism – dominant
inheritance
Disorders
of Melanin Pigmentation
Lack
� generalized
– total albinism
– parcial
albinism
� local
– vitiligo
– leucoderma
Vitiligo
� familial aggregation
� polygenic nature
� association with other
autoimmune diseases (DM,
thyroiditis, gastritis)
� ab against tyrosinase in the serum
� autoreact . T- cellular cytotoxicity
Disorders
of Melanin Pigmentation
Lack
� generalized
– total albinism
– parcial
albinism
� local
– vitiligo
– leucoderma
Leucoderma
� postinflammatory
circumscribed depigmentation
e.g.
– leucoderma syphyliticum
– leucoderma psoriaticum
Disorders
of Melanin Pigmentation
Increase
� generalized– Adison
disease
� local– freckles, nevi
– chloasma /melasma
–melanodermia
–melanoma
Disorders
of Melanin Pigmentation
Increase
� generalized– Adison
disease
� local– freckles, nevi
– chloasma
–melanodermia
–melanoma
Disorders of Phenylalanine
and Tyrosine Metabolism
1. Phenylalanine hydroxylase
PHENYLKETONURIA
2. Homogentisic acid oxydase
ALKAPTONURIA
3. Tyrosinase ALBINISM
Disorders of Phenylalanine
and Tyrosine Metabolism
Phenylalanine Tyrosine
methyl–
acetoacetic
acid
DOPA
MELANINNorepinephrine
Epinephrine
3
1
Homogentisic
acid
Ceroid� features
– light brown
– PAS +
– acidoresistent
– Sudan +-
� origin
– fagocytosis od lipid substances by macrophages
– oxidation of non–saturated lipid acids
Ceroid
� localisation
– places of erythrocytes destruction
– necroses of adipous tissue
– avitamonosis E
– melanosis coli
– Dubin - Johnson syndrome
Lipochrom
� ubiquitous pigment
� exogenous origin
� lipid solvent
� histologically unprovable
Lipofuscin
� features – dark brown
– Sudan +-
– autofluorescence
Lipofuscin
�origin – autophagocytosis
"wear and tear" pigment
from the accumulation
of autophagolysosomes over time.
Lipofuscin
�localisation
– CNS, epithels, muscles, liver
ATROPHIA FUSCA