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Disorders
hshields@bidmc.harvard.edu
:Prepared by
.Dr.Mohammad Shaikhani
Assistant professor
Sulaimani University
College of Medicine
.Dept of Medicine
Skin Findings
Malabsorption
Severe constipation.
Anal fissures.
Argon plasma&embolization.
Hereditary Hemorrhagic Telangiectasia
(Osler–Weber–Rendu Disease):GIT
Pulmonary / cerebral complications including pulmonary
hypertension, paradoxic emboli, air emboli, cerebral abscesses
may come on silently secondary to pulmonary AVMs&need to be
managed proactively.
In patients with pulmonary AVMs, routine antibiotic dental
prophylaxis is recommended for both dental & endoscopic
procedures.
Prophylactic management of the pulmonary AVMs by
embolization or surgical resection should be considered when
AVMs are : 3 mm.
Endothelin-receptor antagonists have been tried.
Hereditary Hemorrhagic Telangiectasia
(Osler–Weber–Rendu Disease):GIT
Patients with hepatic AVMs can present with HF, edema,
increased liver function tests, ascites, variceal bleeding, or
encephalopathy,managed intensively with medical means.
Embolization is used only for palliation given the incidence of
hepatic necrosis.
Invasive procedures as liver biopsy or ERCP should be avoided
given the respective risks for bleeding / sepsis.
When medical management fails, referral for a liver transplant
used with increasing success.
Improvement in nose bleeds/telangiectases with the vascular
endothelial growth factor antagonist, bevacizumab, with
interferon, suggesti trial in HHT AVMs.
Melanoma: case
Erythema nodosum
Avariety of vasculitis.
IBD dermatological features: PG
DD:
Infections: deep fungal infs, bacterial pyodermus, TB.