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Flashcards in Vasculitis & Vascular Lesions Deck (78)
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1

What does the term leucocytoclastic vasculitis mean?

Damage to the vessel wall (caused by immune complex deposition) which results in those vessels leaking out blood and is manifest clinically by Painful Palpable Purpura on the lower legs.

Damage to the vessel wall attracts white cells (leucocytes) which are subsequently become damaged by the immune complex process and break up (-clastic).

2

Describe the pathology of Cryoglobulinemic Vasculopathy.

  • Cryoglobulins are abnormal immunoglobulins that precipitate when it is cold.
  • 3 Different Subtypes.
  • Type 1 - IgM.
  • Type 2 and 3 are mixed (monocloncal and polyclonal) components - and thus are "mixed"
    • Only Type 2 and Type 3 cause vasculitis - in both small and medium sized vessels - hence MIXED.

3

What % of vasculitidies are idiopathic?

50%

4

Is vasculitis a diagnosis?

No

It is a reaction pattern triggered by multiple Exogenous and endogenous timuli.

5

What is the most prevelant paediatric vasculitis?

Henoch-Schonlein Purpura

6

Who gets cutaneous vasculitis and what is it?

All ages and races

Slightly more females more than males.

It refers to any vasculitis that any sized vessels that clinically manifest in the skin.

7

Name some small vessel vasculitides

  • Neutrophilic vasculitis (immune complex mediated)
    • Cutaneous small vessel vasculitis (leukocytoclastic vasculitis)
    • Henoch-Schonlein Purpura
    • Urticarial Vasculitis
  • Eosinophilic Vasculitis - 
    • Hypereosinophilic syndrome
    • Vasculitis associated with connective tissue disease
  • Lymphocytic Vasculitis
    • Perniosis

8

Name some small to medium-sized vessel vasculitides.

  • Neutrophilic vasculitis (immune complex mediated)
    • Cryoglobulinemia
    • Septic vasculitis
  • Eosinophilic Vasculitis 
    • Wegner's Granulomatosis (Granulomatosis with polyangiitis)
    • Churg-Strauss Syndrome (Allergic granulomatous angiitis)
    • Drug Induced ANCA-positive vasculitis
  • Lymphocytic Vasculitis
    • ​Viral & Rickettsial infections
    • Degos disease
    • Vasculitis associated with connective tissue diseases

9

Name some medium to large vessel vasulitidies

  • Neutrophilic vasculitis (immune complex mediated)
    • Polyarteritis nodosa (PAN)
    • Nodular vasculitis
  • Granulomatous Vasculitis - 
    • Giant Cell Arteritis
    • Takayasu arteritis
  • Lymphocytic Vasculitis
    • Kawasaki's disease

10

What are the common causes for cutaneous small vessel vasculitis?

11

What drugs can cause cutaneous small  vessel vasculitis?

  • Allopurinol
  • Antibiotics
  • NSAIDs
  • Thiazides
  • Propylthiouracil

12

What infections can cause cutaneous small  vessel vasculitis?

  • Bacterial
    • Beta-Haemolytic Strep
    • Meningococcus
    • Syphilis
    • Tuberculosis
    • Leprosy
  • Viral
    • URTI
    • Hepatitis

13

Which forms of hepatitis are more likely to form cutaneous small vessel vasculitis?

Hepatitis C > Hepatitis B > Hepatitis A

14

What autoimmune conditions can cause cutaneous vasculitis?

  • SLE
  • RA
  • Sjorgens Syndrome

15

What tumour antigens can cause small vessel cutaneous vasculitis?

  • Lymphoproliferative malignancies
  • Myeloprofilerative malignancies

16

What is the pathology of cutaneous vasculitis?

  • Immune complexes in the vessel wall stimulate the complement cascade.
  • Chemotactic mediators, histamine and pro-inflammatory cytokines are stimulated.
  • ROS (reactive oxygen species) and proteolytic enzymes are released causing fibrinoid necrosis of the vessel wall.

17

Why is a biopsy useful in vasculitis?

  1. It helps to see what vessels are involved
  2. inflammatory cells are deposited in the vessel wall.

18

When should a biopsy be taken and what should be requested with it?

  1. As soon as the cutaneous manifestation occurs - within 48 hours.
  2. Request a DIF - it helps to see what immunoreactants (C3, IgM, IgA, IgA) are being deposited.

19

In what layers of the skin are affected in small vessel vasculitis?

Superficial and mid dermis

20

In what layers of the skin are affected in medium vessel vasculitis?

Deep dermis and subcutis

21

What are the 2 major causes of purpura?

  • Vasculitis
  • Microvascular occlusion syndrome

22

Describe a simple way of diagnosing Purpura?

  • Non-Inflammatory vessel Wall Abnormalities -> NON-PALPABLE PURPURA
    • Collagen Disorders (Ehlers Danlos)
    • Capillary Fragility - amyloidosis, solar purpura, steroid purpura.
  • Inflammatory Vessel Wall Abnormalities or damage due to intravascular thrombi -> PALPABLE PURPURA
    • Vasculitis
    • Pigmented purpura
    • Acute meningitis
    • Thrombi: DIC, Paraproteinemias
  • Coagulation, Platelets and Other Intravascular Abnormalties -> NON-PALPABLE PURPURA
    • Thrombocytopenia
    • Platelet dysfunction
    • Coagulopathies

23

What is the most common manifestation of CSVV (Cutaneous small vessel vasculitis)?

Painful, purpuric macules and papules (palpable purpura)

(They usually occur 1 week after the triggering event)

(Some lesions in the picture form crusted, haemorhagic blisters)

24

How else can CSVV manifest and what does this show about the portion of the skin that is being affected?

  • As haemorrhagic vesicles, pustules or nodules - deep dermis and subcutis (See picture)
  • Livedo reticularis +/- Nodules - deep dermis, subcutis and are often larger vessels being affected. Consider systemic involvement in this scenario.

25

What area of the body most commonly is affected by CSVV and why?

The legs due to hydrostatic pressure and stasis.

26

What are the most common systemic symptoms of patients with vasculitis?

  • Arthralgia
  • Fever
  • Fatigue
  • Malaise
  • System affected
    • Haematuria
    • Parasthesia
    • Abdomina pain.

27

What condition is this?

What is the difference between the phsyiological cause and the secondary cause?

Livedo Reticularis

  • Physiological Livedo Reticularis - due to cold induced vasospasm.
  • Secondary Livedo Reticularis - often due to systemic disease causeing altered blood flow and increased blood viscosity (Medium-sized vessel vasculitides)

28

What condition is this?

What condition is it usually seen in?

Livedo Racemosa

Sneddon's Syndrome (Multiple Stroke Syndrome)

29

Who is more likely to get HSP?

  • Children < 10 years
  • Male>female
  • Winter following an URTI

30

What immunoglobulin is deposited in the vessels wall in HSP?

IgA