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7. Amyloidosis
The deposition of abnormal quantities of amyloid in tissues,
may be primary or secondary and localized or systemic
Fibrous protein consisting of rigid, linear non- branching
aggregated fibrils approx. 7.5 – 10 nn wide & indefinite length
Purpura
Hemorrhage
Thrombosis
TYPES: Abnormal platelet function and sometimes
Gravis-EDS classical features thrombocytopenia is observed
Mitis- Mild expression of classical EDS
Hypermobile- Marked joint laxity 8. Drug Induced Vascular purpuras
Vascular-Severe life-threatening Mechanism includes development of antibody vessel wall
vascular hemorrhage component & development of immune complexes & changes
X-linked-features similar to type Il (different vessel wall permeability
inheritance) Aspirin, warfarin, barbiturates, diuretics, digoxin, methyldopa,
Ocular/Scoliosis- eye problem and several antibiotics
Arthrochalasis- Joint hyperlaxity and Sulfonamides and iodides have been implicated most
congenital dislocations frequently
Periodontal- Dental problems and classical EDS Few petechiae to massive petechial eruptions may vary
Villefranche Berlin Protein Gene Tx: discontinue drug
classification Classificatio Abnormality abnormalit
n y
Classical EDS type I/II Type V collagen COL5A1
9. Others/Miscellaneous
, Scurvy
COL5A2 Insufficient dietary intake of vitamin C (ascorbic acid)
Hypermobility EDS type III Unknown Unknown
Vascular EDS type IV Type III collagen CO3A1 Characterized defective synthesis of collagen &
Kyphoscoliosis EDS type VI Lysyl PLOD1 hyaluronic acid
hydroxylase
deficiency
Symptoms: bleeding of the skin & mucus, gingivitis, rough
Arthrochalasia EDS type Type I collagen COL1A1, / dry skin, joint & body ache, fatigue, depression,
VIIA/B COL2A2
Dermatosparaxi EDS type N-proteinase ADAMST2
susceptible to infection, muscle weakness
s VIIC
EDS: Ehlers-Danlos syndrome
10. Ecchymoses
superficial, bleeding is usually mild, and
laboratory test results are most often normal