Topic 52 Flashcards
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
Hyperuricemia-gout (predisposing factors)
-DM
-alcoholism
-psoriasis
-multiple myeloma
-haemolytic anaemia
-pharmaceutical products (diuretics, chemotherapeutic drugs
aspirin, nicotinicacid)
-carcinoma
-sarcoma
-leukaemia
-lymphoma
-lead poisoning
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)
Hyperuricemia-gout (diagnosis)
- elevated levels of serum uric acid
- X-ray examination (radiolucent urate tophy)
- biopsy from the tophy
Hyperuricemia-gout (differential diagnosis)
- rheumatoid arthritis
- multiple myeloma
- Hand-Schüller-Christian disease
- inflammatory disease of parotid gland
- cancerous disease of the parotid gland
Hyperuricemia-gout (therapy)
- NSAID
- corticosteroids
- analgetics
- diet
Porphyria cutanea tarda-Chronic hepatic porphyria (etiology)
- congenital, erythropoietic form (uncommon)
- hepatic porphyria cutanea tarda (common)
Predisposing factors:
-liver diseases (alcohol)
-iron deficiencies
-effect of drugs
(tetracyclin, chloroquin,
piridoxine)
Porphyria cutanea tarda-Chronic hepatic porphyria (symptoms)
- congenital form:
- reddish-brown discoloration of the teeth
- splenomegaly
- haemolytic anaemy - both form:
- the skin is excessively sensitive to light
- vesicles, bullae, erosions
- ulcerations covered by bloody crusts
- scarring
- alopecia
- periorbital hyperpigmentosis
- hypertrichosis
Porphyria cutanea tarda-Chronic hepatic porphyria (oral symptoms)
-erythema, vesiculo-bullous alterations
(labial mucosa, gingiva, commisures,lips)
-brownish-black haemorrhages
-crusty erosions
-ulcers
-the tongue becomes atrophic erythematous
-candidiasis
Porphyria cutanea tarda-Chronic hepatic porphyria (differential diagnosis)
-pemphigus
-herpetiform dermatitis
-other vesiculobullous
dermatosis
Porphyria cutanea tarda-Chronic hepatic porphyria (therapy)
-Internal and dermatological, local
antiseptic and antimicotic treatment
Hypercarotinaemia-carotene jaundice (etiology)
- carotene accumulated in the blood
- consumption of excess amounts of carrots, eggs, pumpkins, oranges
Hypercarotinaemia-carotene jaundice (symptoms)
-yellowish discoloration of palms, soles, palate
-sclera will not become yellow (distinction from
jaundice)
Hypercarotinaemia-carotene jaundice (differential diagnosis)
-hepatitis
Haemochromatosis-bronze diabetes (etiology)
-genetic disorder of iron metabolism
-haemosiderin from nutrition is accumulated excessively in the liver, kidney, pancreas, inner organs, skin, mucosa
-occours in a secondary manner too:
transfusions, long iron derivate th.
Haemochromatosis-bronze diabetes (pathogenesis)
-liver, heart, pancreas failure may develop
-DM and its complications
-hepatic cirrhosis and its complication
(hepatic carcinoma) may develop
Haemochromatosis-bronze diabetes (symptoms)
Brownish-grey spots containing hemosiderin on
the oral mucosa- not protruding from the level of the surrounding mucosa.
Haemochromatosis-bronze diabetes (diagnosis)
- histopathology
Haemochromatosis-bronze diabetes (differential diagnosis)
- haematoma
- melanoplakia
- malignant lentigo
- amalgam tattoo
- Addison’s disease
Haemochromatosis-bronze diabetes (therapy)
- Blood transfusions
- Deferoxamin injection
Protein deficiency (etiology)
-decreased intake of protein
-malabsorbtion
-metabolic disorder
-chronic disorder
(hepatic or renal)
-malignancies
Protein deficiency (symptoms)
- dry and hyperpigmented skin and hair
- edemas
- muscle wasting
- hypotension
- anaemia
- hypoglicemia
Protein deficiency (oral symptoms)
- atrophic oral mucosa and tongue
- painful inflammation of fungiform papillae
- fissures (rhagades) in the commisure of the lips
- swelling of the parotid gland
Protein deficiency (differential diagnosis)
- vitamin deficiencies (B2,B3)
- anaemia
- candidiasis
Protein deficiency (therapy)
-Protein rich nutrition
