vasculitis and purpuric rashes

Question 1

what is purpura

Answer 1

a non-blanching rash due to blood in skin but outside blood vessels

Question 2

causes for vessel leaking resulting in purpura (5)

Answer 2

1. blood
2. weak connective tissue around bvs
3. trauma to vessels
4. increased pressure in vessels
5. vasculitis (painful palpable purpura)

Question 3

what might medium vessel vasculitis present with (3)

Answer 3

livedo reticularis; retiforma purpura; subcutaneous nodules

Question 4

how might small vessel vasculitis present (4)

Answer 4

1. purpuric macules (non palpable)
2. purpuric papules (palpable)
3. urticarial plaques
4. haemorrhagic vesicles

Question 5

what is retiform purpura

Answer 5

livedo reticularis with purpura

Question 6

causes for non-inflammatory retiform purpura (8)

Answer 6

1. platelet plugs
2. cold gelling
3. organisms (apergillus etc.)
4. coagulation (proetin C/S deficiency, warfarin necrosis etc.)
5. vasular coagulopathy
6. embolism/cholesterol blockage
7. RBCs (sickle cell, malaria)
8. other (spider bite, calciphylaxis)

Question 7

what contition is part of the vasculitis spectrum

Answer 7

urticaria - mild vasculitis

Question 8

causes for inflammatory retiform purpura

Answer 8

1. medium vessel vasculitis
2. vessel occulsion/constriction/inflammation e.g. septic vasculitis, chilblains

Question 9

what associated conditions are seen in GPA vasculitis

Answer 9

red eye, eye pain, ear pain, deafness, oral/nasal ulcers

Question 10

what associated conditions are seen in EGPA vasculitis

Answer 10

asthma, sinus congestion

Question 11

when can a vasculitic lesion be biopsied

Answer 11

only within 48hrs of presentation - after this changes occur that make diagnosis hard for the pathologists, can be confused with vessel occlusion

Question 12

chapel hill classification

Answer 12

classification for vasculitis (large, medium, small etc.)

Question 13

what might trigger cuteanous small vessel vasculitis and what do you need to look out for

Answer 13

trigger - often idiopathic but can be triggered by drugs, infection, malignancy;
need to look out for progression of disease, often a precursor to systemic disease

Question 14

erythema elevatum diutinum presentation

Answer 14

red/brow/violet plaques over buttocks, achilles, dorsa of hands and knees

Question 15

erythema elevatum diutinum associated conditions

Answer 15

autoimmune - coeliacs, diabetes, IBD;
infections - HIV, hepatitis, syphilis;
haem malignancy - myeloma, IgA, gammopathies

Question 16

granuola faciale presentation

Answer 16

soft, red-brown plaques or nodules on the faces of 40-60yro

Question 17

granuola faciale treatment

Answer 17

tacrolimus or intralesional steroids

Question 18

what is IgA vasculitis (henoch schonlein purpura)

Answer 18

IgA deposits in small vessels of skin and GI tract, commonly presents with arthritis

Question 19

IgA vasculitis treatment

Answer 19

supportive treatment and systemic steroids (esp if renal involvement)

Question 20

what is cryoglobulinaemic vasculitis

Answer 20

cryoglobulins desposited as immune complexes triggering vasculitis

Question 21

cryoglobulinaemic vasculitis presentation

Answer 21

myalgia, fever, headache, weakness, weightloss, arthralgia purpura, tiggered by cold

Question 22

what is hypocomplementaemic urticarial vasculitis

Answer 22

a rare form of vasculitis characterized by inflammation of the small blood vessels and low levels of complement proteins in the blood - separate from urticarial vasculitis; it is highly associated w SLE and may test +ve for anti-C1q antibodies

Question 23

hypocomplementaemic urticarial vasculitis associated symptoms

Answer 23

arthritis, glomerulonephritis, pulmonary and ocular disease, angioedema, arthralgia, abdominal pain

Question 24

hypocomplementaemic urticarial vasculitis treatmetn

Answer 24

similar to SLE - systemic steroids, hydroxychloroquine, dapsone, colchicine

Question 25

what is microscopic polyangiitis

Answer 25

a necrotising vasculitis mainly affecting small vessels; P-ANCA

Question 26

MPA presentation

Answer 26

palpable purpura with necrosis, mouth ulcers and splinter haemorrhages; fever, weightloss and myalgia may be seen, pulmonary and renal involvement are common

Question 27

MPA management

Answer 27

prednisolone, cyclophosphamide, rituximab to induce remission; maintenance with azathioprine

Question 28

GPA presentation

Answer 28

palpable purpura; tender subcutaneous nodules, vesicles and petechiae; non-specific uclers or pyoderma gandrenosum and oral ulceration; saddle nose

Question 29

what is EGPA

Answer 29

eosiniophilic rich granulomatous inflammation, often seen alongside asthma and other pulmonary involvements

Question 30

EGPA presentation

Answer 30

palpable purpura and inflitrated nodules (usually on scalp/limbs); necrotisinf livedo; migratory erythema; new onset Raynaud’s; aseptic pustules or vesicles

Question 31

what is cutaneous polyarteritis nodosum (PAN)

Answer 31

a form of single organ vasculitis of the skin - no systemic involvement -> different to non-ANCA associated PAN which involved GI, heart, kidneys etc.

Question 32

what could trigger PAN

Answer 32

infection or drugs

Question 33

investigations for PAN

Answer 33

deep incisional biopsies on medium sized arteries; CT angiography may be helpful in systemic PAN

Question 34

cutaneous PAN treatement

Answer 34

NSAIDs or pred/azathioprine

Question 35

what is Kawasaki disease

Answer 35

acute illness of infants and young children characterized by symptoms such as fever, rash, and glandular swelling, associated with a vasculitis that particularly affects the coronary arteries and may result in serious heart disease

Question 36

Kawasaki disease presentation

Answer 36

spiking fever unresponsive to paracetemol; acral/perianal erythema; strawberry tongue; uveitis; conjunctivitis; fissured lips; lumphadenopathy