Topic 52 Flashcards
(48 cards)
Acrodermatitis enteropathica (etiology)
- autosomal recessive hereditary disease
- caused by zinc deficiency
- in infancy and childhood untreated may be lethal - in adulthood due to :
- parenteral feeding
- malabsorbtion
- inflammatory bowel diseases
- chronic alkcoholism
- hepatic diseases
- special diets
- anorexia nervosa
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)
Acrodermatitis enteropathica (differential diagnosis)
- psoriasis
- candidiasis
- eczema
- perioral dermatitis
- bullous epidermolysis
Acrodermatitis enteropathica (therapy)
- Zinc substitution
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
Amyloidosis (clinical forms)
- primary (systemic/localized)
- secondary (systemic/localized)
- familial
- senile
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
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
Amyloidosis (diagnosis)
- histological examination (obtained from biopsy)
- Congo red, thioflavine+ stains
Amyloidosis (differential diagnosis)
- scleroderma
- myxoedema
- sarcoidosis
Amyloidosis (therapy)
symptomatic treatment
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
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
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
Lipoid proteinosis-hyalinosis of the skin and mucosae-Urbach-Wiethe disease (diagnosis)
- hystopathology
Lipoid proteinosis-hyalinosis of the skin and mucosae-Urbach-Wiethe disease (differential diagnosis)
- amyloidosis
- scleroderma
- porphyria
Lipoid proteinosis-hyalinosis of the skin and mucosae-Urbach-Wiethe disease (therapy)
- supportiv therapy
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)
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
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
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
Waldenström’s macroglobulinaemia (differential diagnosis)
- Werlhov’s disease
- amyloidosis
- Osler’s disease
Waldenström’s macroglobulinaemia (therapy)
- plasmaferesis
- transfusions
- the prognosis is poor
Hyperuricemia-gout (etiology)
- accumulation of uric acid
- commonly caused by familial factors
