VESSEL WALL ABNORMALITIES
Types of vessel wall abnormalities :
1. Hereditary: HHT, EDD, Osteogenesis imperfecta
2. Inflammatory: Bacterial, Viral, Vasculitis.
3. Abn. Collage: Senile, Scurvy, Steroids.
4. Metaboli : Liver dis, Renal, Amyloidosis.
5. Others: Factitious, Paraproteinaemia, Drugs.
Hereditary Hemorrhagic Telangiectasia (HHT)
(Osler – Rendu –Weber synd.)
A.D., Telengiectasia and small aneurysms on Fingers, lips, Face,
Tongue, Nasal passages, Lungs, G.I.T. / Usually appear at adulthood.
Signif. No. have Pul. A.V. malformation (PAVMS) → Arterial
Hypoxaemia due to Rt. To It. Shunt → paradoxical embolism →
stroke or cerebral abscess.
Recurrent Bleeding e.g. Epistaxis or Malaena → Fe def. An.
Deficult bec. Of multiple Bleeding sites.
Regular Fe therapy
Local Cuatary or Laser, Oestrogens
Ehlers – Donalos Dis :
AD. , Collagen defect
Fragile Bl. Ves. And organ memb. → Bleeding and organ rupture
. Vit C def. → affect normal synthesis of collagen
. Perifollicular and patechial haemorrhage, sub‐ periosteal bleeding
. Diagnosis : Dietary History
Henoch – Schonlein Purpura (HSP)
. Children and young adults .
. Immune complex deposition in small ves. With vasculitis .
Often preceded by upper resp. inf.
1. Purpura: (raised) Buttocks and legs
2. Abdominal colicky pain and bleeding
3. Arthritis : Knees, Ankles .
4. Nephritis : 40% (May be later – 4 wks.)
Diagnosis : Clinical, Tissue biopsy ( IgA deposition in bl. Ves.)
Prognosis : good, Adverse if HTN, RF, Proteinuria > 1.5 g / day
Steroids for joint or GI involvment
Nephritis – pulse iv steroids ± immunosup.