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Flashcards in Topic 52 Deck (48)
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1

Acrodermatitis enteropathica (etiology)

1. autosomal recessive hereditary disease
- caused by zinc deficiency
- in infancy and childhood untreated may be lethal
2. in adulthood due to :
-parenteral feeding
-malabsorbtion
-inflammatory bowel diseases
-chronic alkcoholism
-hepatic diseases
-special diets
-anorexia nervosa

2

Acrodermatitis enteropathica (symptoms)

-acral dermatitis
-periorificial dermatitis
-alopecia
-diarrhea
-retardation of growth
-prolonged wound healing
-predisposition to infections
-peeling of hans and soles
-paronychia
-photophobia
-psychic disorder
-vesicles, bullae, pustules
on the basis of erythema
-crusty, scaly psoriasis after a couple of
days
-bacterial and fungal symptomes
(nose, mouth, anal and genital regions)
-around mouth: pustules similar to
impetigo on an erythema
-ulcerativ fissural angular cheilitis
(perleche)
-edema and erythemas on the mucosa
of the lips and the oral cavity
-aphtous ulcers
-whitish pseudomembranous
candidiasis (buccal mucosa and
tongue)

3

Acrodermatitis enteropathica (differential diagnosis)

-psoriasis
-candidiasis
-eczema
-perioral dermatitis
-bullous epidermolysis

4

Acrodermatitis enteropathica (therapy)

- Zinc substitution

5

Amyloidosis (etiology)

-overproduction of
immunglobulines
due to disturbances of
protein metabolism
-amyloid (a chemical compound made of immunglobulines and carbohydrates)
-deposits within the skin
-in the subcutaneous layers
-gastrointestinal tract
-inner organs
-bones
-nerves
-vessels
-muscles
Predisposing fastors:
-rheumatoid arthritis
-multiple myeloma

6

Amyloidosis (clinical forms)

- primary (systemic/localized)
- secondary (systemic/localized)
- familial
- senile

7

Primary Systemic Amyloidosis (symptoms)

Primary systemic amyloidosis:
-periorbital and perinasal transparent papules
-macroglossia
-amyloid deposits (muscles- tongue)
-enlarged tongue, hard to touch, retains of the teeth
-reddish-yellowish nodules appear on lateral sides of the tongue
-atrophy of lingual papillae, purpuras
-petechiae, purpuras, ecchymoses („pinch purpuras”)
-haemorrhagic bullae
-swollen minor and major salivary glands
-regional swelling of lymphatic glands
-xerostomia
-wax-like yellow skin
-within the oral cavity: dark, reddish tone

8

Secondary Systemic Amyloidosis (symptoms)
accompanying chronic diseases:
- Hodgkin’s disease
- tuberculosis
- rheumatoid arthritis
- regional ileitis

-gingival swelling (apart from macroglossia)
-nodules on mucosa
-plaques, papules, bullae

9

Amyloidosis (diagnosis)

-histological examination (obtained from biopsy)
-Congo red, thioflavine+ stains

10

Amyloidosis (differential diagnosis)

-scleroderma
-myxoedema
-sarcoidosis

11

Amyloidosis (therapy)

symptomatic treatment

12

Lipoid proteinosis-hyalinosis of the skin and mucosae-Urbach-Wiethe disease (pathogenesis)

- unknown etiology
- hyalin-like lipoprotein is deposited within tissue
layers of GI, respiratory tract, skin, mucosa

13

Lipoid proteinosis-hyalinosis of the skin and mucosae-Urbach-Wiethe disease (symptoms)

-in childhood: hoarseness
-yellowish-whitish papules
on the eyelids
-rigid and mottled facial skin
-papules, nodules, pustules and later scars on the skin

14

Lipoid proteinosis-hyalinosis of the skin and mucosae-Urbach-Wiethe disease (oral symptoms)

- nodular, papular, whitish, yellowish on buccal
mucosa, tongue, lips, palate
- streak like, reddish-yellow, drip-like hyalinization
of gingiva
- atrophic, hard, enlarged tongue
- short, thick frenulums (partial immobilization)
- hyalin deposits become harder and resemble scars
- oral infections and ulcers
- obstruction of the parotid passage
- reoccuring painful swelling of the parotid gland
- dental enamel hypoplasia, hypodontia

15

Lipoid proteinosis-hyalinosis of the skin and mucosae-Urbach-Wiethe disease (diagnosis)

- hystopathology

16

Lipoid proteinosis-hyalinosis of the skin and mucosae-Urbach-Wiethe disease (differential diagnosis)

- amyloidosis
- scleroderma
- porphyria

17

Lipoid proteinosis-hyalinosis of the skin and mucosae-Urbach-Wiethe disease (therapy)

- supportiv therapy

18

Waldenström’s macroglobulinaemia (etiology)

-chronic, often lethal disease
-charactized by the overproduction of IG-s
(gammopathy)
-proliferation of pathological
lymphoid cells
(hybrid cells with features of
B and plasma cells)

19

Waldenström’s macroglobulinaemia (pathogenesis)

-macroglobulines create a complex with blood clotting factors
-macroglobulines linked to the surface of erythrocytes
-damage the capillary wall
-cause hemophilia or hemolytic anaemia

20

Waldenström’s macroglobulinaemia (symptoms)

-mainly by elderly men
-purpuras all over the body
-dypnoea
-loss of weight
-fatigue
-hepato-splenomegaly
-anaemi
-thrombocytopenia
-granulocytopenia
-nasal and oral mucosa
bleedings

21

Waldenström’s macroglobulinaemia (oral symptoms)

-petechiae, ecchymoses, sugillations in
groups or extended clusters,
-may meld to each other: finally become
ulcerative and painful
-gingival hemorrhage
-prolonged bleeding after extraction
Rarely:
-swelling of salivary glands and
xerostomia
- generalized lymphadenopathy
-increased predisposition for thrombosis

22

Waldenström’s macroglobulinaemia (differential diagnosis)

- Werlhov’s disease
- amyloidosis
- Osler’s disease

23

Waldenström’s macroglobulinaemia (therapy)

-plasmaferesis
-transfusions
-the prognosis is poor

24

Hyperuricemia-gout (etiology)

-accumulation of uric acid
-commonly caused by familial factors

25

Hyperuricemia-gout (predisposing factors)

-DM
-alcoholism
-psoriasis
-multiple myeloma
-haemolytic anaemia
-pharmaceutical products (diuretics, chemotherapeutic drugs
aspirin, nicotinicacid)
-carcinoma
-sarcoma
-leukaemia
-lymphoma
-lead poisoning

26

Hyperuricemia-gout (symptoms)

-arthritis (joint of leg: ankles, knee) pain, accompanied with minor swelling
-tophi various sizes appear on the hands and legs (deposition of urate crystals within
periarticular tissue layers)
-urate deposition on skin, cartilage, bones,
inner organs (kidneys- gout associated nephropathy)
-bilateral swelling of the parotid gland
(sialadenosis)

27

Hyperuricemia-gout (diagnosis)

-elevated levels of serum uric acid
-X-ray examination (radiolucent urate tophy)
-biopsy from the tophy

28

Hyperuricemia-gout (differential diagnosis)

-rheumatoid arthritis
-multiple myeloma
-Hand-Schüller-Christian disease
-inflammatory disease of parotid gland
-cancerous disease of the parotid gland

29

Hyperuricemia-gout (therapy)

-NSAID
-corticosteroids
-analgetics
-diet

30

Porphyria cutanea tarda-Chronic hepatic porphyria (etiology)

1. congenital, erythropoietic form (uncommon)
2. hepatic porphyria cutanea tarda (common)
Predisposing factors:
-liver diseases (alcohol)
-iron deficiencies
-effect of drugs
(tetracyclin, chloroquin,
piridoxine)