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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. Pigments. Definition: colored substances in the organism or environment. Pigments. Classification: endogenous

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general pathology
General Pathology

Basic Principles of Cellular and Organ Pathology

Autogenous Pigments

Jaroslava Dušková

Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague

pigments
Pigments

Definition:

colored substances

in the organism

or environment

pigments3
Pigments

Classification:

  • endogenous
      • autogenous
      • hemoproteins derived
  • exogenous
autogenous pigments
Autogenous Pigments

-color substances formed in the organism as metabolism products

  • melanin
  • ceroid
  • lipofuscin
melanin
Melanin
  • oculocutaneous

(origin from tyrosine in melanocytes)

  • neuromelanin - subst. nigra

(origin from dopamin)

melanin types
Melanin - types
  • eumelanin – insoluble , brown-black
  • phaeomelanin – soluble, yellow-red (high sulphur content)
melanin production
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
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
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
Melanin - Features
  • brown
  • destained with H2O2
  • reducing AgNO3
disorders of melanin pigmentation
Lack

generalized

total albinism

parcial albinism

local

vitiligo

leucoderma

Disorders of Melanin Pigmentation
albinism
Albinism
  • autosomal recessive heredity
    • tyrosinase deficiency
    • tyrosinase positive – melanosomes defect
  • oculo-cutaneous albinoidism – dominant inheritance
disorders of melanin pigmentation13
Lack

generalized

total albinism

parcial albinism

local

vitiligo

leucoderma

Disorders of Melanin Pigmentation
vitiligo
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 pigmentation15
Lack

generalized

total albinism

parcial albinism

local

vitiligo

leucoderma

Disorders of Melanin Pigmentation
leucoderma
Leucoderma
  • postinflammatory circumscribed depigmentation e.g.
    • leucoderma syphyliticum
    • leucoderma psoriaticum
disorders of melanin pigmentation17
Increase

generalized

Adison disease

local

freckles, nevi

chloasma /melasma

melanodermia

melanoma

Disorders of Melanin Pigmentation
disorders of melanin pigmentation18
Increase

generalized

Adison disease

local

freckles, nevi

chloasma

melanodermia

melanoma

Disorders of Melanin Pigmentation
disorders of phenylalanine and tyrosine metabolism
Disorders of Phenylalanine and Tyrosine Metabolism

1. Phenylalanine hydroxylase PHENYLKETONURIA

2. Homogentisic acid oxydase

ALKAPTONURIA

3. Tyrosinase ALBINISM

disorders of phenylalanine and tyrosine metabolism20
Disorders of Phenylalanine and Tyrosine Metabolism

Phenylalanine

Tyrosine

1

3

Homogentisic

acid

DOPA

2

cc

methyl–

acetoacetic

acid

Norepinephrine

Epinephrine

MELANIN

ceroid
Ceroid
  • features
    • light brown
    • PAS +
    • acidoresistent
    • Sudan +-
  • origin
    • fagocytosis od lipid substances by macrophages
    • oxidation of non–saturated lipid acids
ceroid22
Ceroid
  • localisation
    • places of erythrocytes destruction
    • necroses of adipous tissue
    • avitamonosis E
    • melanosis coli
    • Dubin - Johnson syndrome
lipochrom
Lipochrom
  • ubiquitous pigment
  • exogenous origin
  • lipid solvent
  • histologically unprovable
lipofuscin
Lipofuscin
  • features
    • dark brown
    • Sudan +-
    • autofluorescence
lipofuscin25
Lipofuscin
  • origin
    • autophagocytosis

"wear and tear" pigment

from the accumulation

of autophagolysosomes over time.

lipofuscin26
Lipofuscin
  • localisation
    • CNS, epithels, muscles, liver

ATROPHIA FUSCA