Tuesday 1 September 2015

Felty's syndrome- Infections in rheumatoid arthritis

Felty's syndrome is characterised by triad of rheumatoid arthritis, splenomegaly and neutropenia. There is a risk of recurrent infections due to neutropenia. Patient may also have anemia and thrombocytopenia because of hypersplenism. Best treatment for Felty's syndrome is to control underlying rheumatoid disease.

Patient with deforming RA found to have splenomegaly

Moyamoya disease- An uncommon cause of stroke

Moyamoya disease is a progressive, occlusive disease of the cerebral vessels with particular involvement of the circle of Willis and the arteries that form it. Collateral vessels develops around the blocked artery to compensate for the blockage, but the collateral vessels are small, weak, and prone to hemorrhage, aneurysm and thrombosis. On conventional X-ray angiography, these collateral vessels have the appearance of a "puff of smoke" for which it is named as Moyamoya (Moyamoya is term for puff of smoke in japanese). It can present as migraine type headache, recurrent transient ischemic attacks (TIAs), stroke or hemorrhage in the brain. Therapy is primarily directed at the complications of the disease.

Rich collaterals around the blocked artery giving the appearance of puff of smoke.

Gianotti-crosti syndrome : An atypical presentation of Hepatitis B virus infection

In children, Hepatitis B may present rarely with anicteric hepatitis, a non-pruritic papular rash on the face, buttocks and limbs, and lymphadenopathy. This is known as papular acrodermatitis of childhood or Gianotti- Crosti syndrome.  Likely pathogenesis for this condition is local type IV hypersensitivity reaction to offending viral antigen within the dermis. It is more common among children with atopic dermatitis. With the advent of global HBV vaccination, EBV is now more common pathogen for this uncommon disorder. It is a benign self limited condition which requires no treatment.
Child with acute hepatitis B infection with non pruritic papular rash