Purpura

Question 1

Hypergammaglobulinaemic Purpura of Waldenstrom pathogenesis

Answer 1

• associated with hypergammaglobulinaemia
• presence of small circulating immune complexes containing IgG or IgA RF –> these are really soluble so might explain the rapdiity which lesions appear and resolve
• increased development of autoimmune and lymphoproliferative disorders

Question 2

Hypergammaglobulinaemic Purpura of Waldenstrom clinical

Answer 2

• slight itch, mild tingling, burning
• may be aggravated by tight-fitting garments, prolonged standing and heat
• macular haemorrhage on lower extremities, these can be fleeting
• sometimes palpable purpura
• can be:
  
  • Primary: young patients
  • Secondary: autoimmune connective tissue disease (Sjogren or RA or lupus), or rarely a monoclonal gammopathy, lymphoma or multiple myeloma
• Investigations: polycloncal hypergammaglobulinaemia and elevated ESR
  
  • standard RF assays only check IgM so this will come back normal, you need to order the IgG and IgA
  • Anti-SSA/Ro and SSB/La are often positive

Question 3

Hypergammaglobulinaemic Purpura of Waldenstrom treatment

Answer 3

• avoid precipitating factors: alcohol, prolonged standing
• used to use support stockings and aspirin but that can actually flare it
• ?colchicine

Question 4

Gardner Diamond clinical

Answer 4

• Psych: depression, anxiety, difficulties handing aggression and hostility, hypochondriacs, etc
• Sudden onset of painful, swollen bruises of variable size in any area of the body
• resolve within 2 weeks, and then recur
• reports of injection of own blood brings it on

Question 5

Gardner Diamond treatment

Answer 5

rx: psych management, very difficult to treat

Question 6

Mondor Syndrome of Superficial Thrombophlebitis pathogenesis

Answer 6

• pathogenic factors: trauma, excessive physical activity, surgery, mastitis, breast abscess, pendulous breasts
• 10% have underlying breast carcinoma
• can be associated with rheumatoid arthritis, filariasis, pregnancy, OCP, IVDU, IV catheters, other malignancies, hypercoagulable states

Question 7

Mondor Syndrome of Superficial Thrombophlebitis clinical

Answer 7

• sudden onset of chest pain with one or more visible or palpable cords
• associations: tenderness, sensation of tension, erythema, ecchymosis, pruritis, arthralgia, fever
• worsens with pulling the skin or elevating the breast and ipsilateral extremity
• is unilateral
• F>M

Question 8

Mondor Syndrome of Superficial Thrombophlebitis treatment

Answer 8

• and self limited, except when associated with breast cancer
• rate of recurrence ~5%
• NSAIDs, LMWH
• superficial venous thrombosis involving the main trunk of the saphenous bein has the strongest associated with VTE: if near the sapheno-femoral or sapheno-popliteal it needs to be removed

Question 9

Papular purpuric gloves and socks syndrome

Answer 9

Viral rash resulting in painful redness and swelling of the feet and hands. Most common cause parvovirus B19, EVB
Is rapidly progressive
Can result in polyarthropathy, aplastic crises, intrauterine death