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Dermatology Diploma > Vasculitis & Vascular Lesions > Flashcards

Vasculitis & Vascular Lesions Flashcards

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1
Q

What does the term leucocytoclastic vasculitis mean?

A

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).

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2
Q

Describe the pathology of Cryoglobulinemic Vasculopathy.

A
  • 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.
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3
Q

What % of vasculitidies are idiopathic?

A

50%

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4
Q

Is vasculitis a diagnosis?

A

No

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

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5
Q

What is the most prevelant paediatric vasculitis?

A

Henoch-Schonlein Purpura

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6
Q

Who gets cutaneous vasculitis and what is it?

A

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.

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7
Q

Name some small vessel vasculitides

A
  • 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
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8
Q

Name some small to medium-sized vessel vasculitides.

A
  • 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
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9
Q

Name some medium to large vessel vasulitidies

A
  • Neutrophilic vasculitis (immune complex mediated)
    • Polyarteritis nodosa (PAN)
    • Nodular vasculitis
  • Granulomatous Vasculitis -
    • Giant Cell Arteritis
    • Takayasu arteritis
  • Lymphocytic Vasculitis
    • ​Kawasaki’s disease
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10
Q

What are the common causes for cutaneous small vessel vasculitis?

A
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11
Q

What drugs can cause cutaneous small vessel vasculitis?

A
  • Allopurinol
  • Antibiotics
  • NSAIDs
  • Thiazides
  • Propylthiouracil
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12
Q

What infections can cause cutaneous small vessel vasculitis?

A
  • Bacterial
    • Beta-Haemolytic Strep
    • Meningococcus
    • Syphilis
    • Tuberculosis
    • Leprosy
  • Viral
    • URTI
    • Hepatitis
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13
Q

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

A

Hepatitis C > Hepatitis B > Hepatitis A

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14
Q

What autoimmune conditions can cause cutaneous vasculitis?

A
  • SLE
  • RA
  • Sjorgens Syndrome
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15
Q

What tumour antigens can cause small vessel cutaneous vasculitis?

A
  • Lymphoproliferative malignancies
  • Myeloprofilerative malignancies
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16
Q

What is the pathology of cutaneous vasculitis?

A
  • 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.
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17
Q

Why is a biopsy useful in vasculitis?

A
  1. It helps to see what vessels are involved
  2. inflammatory cells are deposited in the vessel wall.
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18
Q

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

A
  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.
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19
Q

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

A

Superficial and mid dermis

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20
Q

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

A

Deep dermis and subcutis

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21
Q

What are the 2 major causes of purpura?

A
  • Vasculitis
  • Microvascular occlusion syndrome
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22
Q

Describe a simple way of diagnosing Purpura?

A
  • 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
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23
Q

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

A

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)

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24
Q

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

A
  • 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.
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25
Q

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

A

The legs due to hydrostatic pressure and stasis.

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26
Q

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

A
  • Arthralgia
  • Fever
  • Fatigue
  • Malaise
  • System affected
    • Haematuria
    • Parasthesia
    • Abdomina pain.
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27
Q

What condition is this?

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

A

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)
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28
Q

What condition is this?

What condition is it usually seen in?

A

Livedo Racemosa

Sneddon’s Syndrome (Multiple Stroke Syndrome)

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29
Q

Who is more likely to get HSP?

A
  • Children < 10 years
  • Male>female
  • Winter following an URTI
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30
Q

What immunoglobulin is deposited in the vessels wall in HSP?

A

IgA

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31
Q

Describe the Triad of symptoms/signs in HSP.

A
  1. Abdominal pain
  2. Arthralgia/arthritis
  3. Haematuria/Proteinuria due to nephritis

Other symptoms: fever, malaise, fatigue.

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32
Q

How is HSP Managed?

A

By GP with weekly BP & MSU sticks for the first 4 weeks

If HTN, macroscopic haematuria or proteinuria then refer to paediatrics.

See Royal Berkshire Guidance

http://www.royalberkshire.nhs.uk/Downloads/GPs/GP%20protocols%20and%20guidelines/Paediatric%20handbook/Henoch%20Schonlein%20Purpura%20guideline.pdf

33
Q

In urticarial vasculitis, what autoimmune conditions is it strongly associated with?

A
  • SLE
  • Sjorgen’s Syndrome
34
Q

How are urticarial lesions in UV (urticarial vasculitis) different from classic urticaria?

A

They are less itchy and more painful and burning.

35
Q

What is the disease course in UV?

A
  • Urticarted lesions persist for more than 24 hours
  • Resolve with hyperpigemnted / bruised appearance
36
Q

What is the treatment for UV?

A

Antihistmaines + A short dose of systemic steroids.

If recalcitrant, consider immunosuppressives or biologics.

37
Q

What are the extracutaneous manifestations of UV?

A

Arthralgia of the hands, feet and ankles.

Sometimes pulmonary and renal involvement.

38
Q

What are cryoglobulins?

Which type of Cryoglobulins cause cryglobulinemic vasculopathy?

A

Abnormal globulins that precipitate in cold environments.

There are 3 types

  • Type 1 - monoclocal IgM
  • Type 2 and 3 are mixed - these cause “mixed” and small and medium-sized vessel inflammation.
39
Q

What patients get cryoglobinemic vasculopathy?

A
  • Hepatitis C (50% will have cryoglobins but only 5% will have vasculopathy)
  • Autoimmune disease
    • RA
    • Sjorgens Syndrome
    • Systemic Sclerosis
  • Lymphoproliferative disorders
40
Q

What are the clinical manifestations of Cryoglobulinemia?

A

They ultimately reflect the depth of the disease.

  • Palpable purpura (Dermal and small vessel)
  • Livedo Reticularis (Subcutis and medium vessel)
  • Ulceration
  • Necrosis
41
Q

What is PAN and who gets it?

A

Polyarteritis Nodosa

  • Middle aged adults
  • Usually males
  • Cutaneous PAN is more common in children.
42
Q

What causes Polyarteritis Nodosa (PAN)?

A
  • Viruses
    • HBC
    • HCV
    • Strep
    • Parvovirus
  • Inflammatory diseases
  • Malignany
  • Drugs.
43
Q

What does PAN look like clinically?

A
  • Livedo Racemosa
  • Retiform Pupura
  • Purpuric cutaneous nodules and punched out ulcers.
44
Q

What are complications in PAN?

A
  • Mylagia
  • Arthralgia
  • Mononeuritis
  • Dyspnoea due to congestive heart failure
  • Renovascular complications.
45
Q

What is the treatment for PAN?

A
  • Systemic steroids
  • Cytotoxic agents (Cyclophosphamide)
  • NSAIDs for pain relief
46
Q

What is the treatment for cutaneous PAN?

A

Intralesional steroids

47
Q

In PAN, what cutaneous manifestation is this?

A

Retiorm Pupura

On the dorsal foot.

48
Q

In PAN, what cutaneous manifestation is this?

A

Livedo racemosa

of the legs. A sign of medium-sized vessel disease.

49
Q

What is Pityriasis Lichenoides et Varioliformis Acuta? (PLEVA)

A

It is a lymphocytic variant of small vessel vasculitis.

50
Q

Why is it mistaken for Chicken pox?

How is it different?

A

Both have crosps of crusted vesicular lesions.

PLEVA will taken months to go away whereas chickenpox takes days.

PLEVA will also heal with varioliform scars.

51
Q

What is the treatment for PLEVA?

A
  1. Topical Corticosteroids + Phototherapy
  2. Tetracyclines for their anti-inflammatory properties
  3. If children - consider 6 week trial of Erythromycin.
52
Q

What is Pigmented Purpuric Dermatoses/Capillaritis?

A

Mild inflammation of the papillary dermal capillaries resulting in extravasation of red blood cells into the skin with marked hemosiderin deposition.

53
Q

Describe the algorithmic approach to take to vasculitis.

A
54
Q

How does cone distinguish Capillaritis/Pigmented Purpuric Dermatoses from CSVV or other vasculitides?

A
  1. Background of light brownish discolouration due to hemosiderin.
  2. No evidence of coagulopathy or systemic disease.
  3. Lesions are more commonly on the lower limbs
  4. It is more common in young people.
55
Q

What is the treatment of Capillaritis?

(Picture shows pigemnted Purpua of the axilla with petechial haemorrhage and light brown hemosiderin deposition)

A

No proven treatments

Short term topical corticosteroids.

Ascorbic acid

Compression Hosiery.

Self limiting or Persisting/Relapsing.

56
Q

What is more likely to be caused by drugs, Erythema Multiforme or Stevens-Johnson Syndrome?

A

Steven-Johnson’s Syndrome

57
Q

Why is Erythema Multiforme called Multiforme?

A

Because there are MULTIPLE manifestations on the skin.

  • Mild Maculopapular target lesions
  • Severe haemorrhagic bullae.
58
Q

What parts of the body are most affected in EM?

Is onset slow or fast?

A

Upper limbs > Lower limbs

Fast - the crops appear over a few days.

59
Q

Do EM lesions show a Koebner phenomenon?

A

Yes they do.

60
Q

What mucosal surfaces are usually affected?

A

Oral > Genital > Ocular

61
Q

What is the difference between EM minor and major?

A

EM Minor = no systemic or mucosal involvement.

EM Major - mucosal and systemic involvement

62
Q

What is the treatment for Erythema Minor?

A
  • Symptomatic treatment
    • Topical steroids
    • Oral care
63
Q

What is the treatment for Erythema Major?

A
  • Admission with good nursing care.
  • Treatment in a burns unit is useful due to fluid loss and electrolytie imbalances.
  • Oral steroids are still given despite limited evidence.
    • 30-60mg/day.
64
Q

In recurrent EM, what is a treatment option?

A

Acyclovir for 6 months - 200mg TDS or 400mg BD

65
Q

What is the treatment in severe cases?

A
  • In severe cases, empiric treatment with dapsone, thalidomide, azathioprine or PUVA are possible options.
  • IV Immunoglobulin therapy is still in development.
66
Q

Congenital Vascular Lesions

What is this?

A

Capillary Haemangioma

67
Q

Congenital Vasular Lesions,

What is this?

A

Ulcerated Capillary Haemangioma

68
Q

Congenital Vascular Lesions

What is the difference between a vascular malformation and a haemangioma?

A
  • Haemangiomas - benign neoplasms involving a proliferation of the endothelial lining of capillary walls.
    • They are not prsent at birth and arise in the first few months of infancy.
  • Vascular malformations - present at birth. Are malformations of capillaries, veins, arteries or lymphatics.
69
Q

In congenital coagulopathies, who should be referred to paediatrics?

A
  • All lesions around the eyes
  • Genital areas
  • Ulcerated lesions
  • Parental anxiety
  • Midface lesions (these may be part of FACE syndrome - associated with cardiac abnormalities)
70
Q

If a lesion is over the spine, why is that a worry and what should you do?

A

50% area linked to underlying problems.

  • Tethering or compression of spinal cord
  • External genital abnormalities

Do an MRI scan

71
Q

What is the management of capillary haemangiomas/strawberry naevi?

A
  • Topical Beta-Blockers - Timolol 0.5% Solution (good for superficial)
72
Q

What is this?

A

Port wine stain

Malformations of developmental origin present at birth. The ectasia of superficial dermal capillaries malform.

They never involute and go away.

73
Q

What 2 conditions are Port Wine Stains related to?

A
  • Sturge-Weber Syndrome - Intracranial angiomas, epilepsy, mental retardation, gluacoma.
  • Klippel-Trenaunay-Weber Syndrome - lim hypertrophy with soft tissue and bone overgrowth, varicose veins, developmental defects and high rate of DVT and PE.
74
Q

What is the treatment for Port Wine Stains?

A

Specialist dermatologist with access to lasers and cosmetic camouflage.

75
Q

Aquired vascular lesions

What is this?

A

Venous Lake

Common in the elderly and from dilated blood vessels.

Purely cosmetic.

76
Q

Aquired vascular lesions

What is this?

A

Glomus Tumour

Benign Vascular neoplasms

In young adults.

Small red-blue papules in the dermis or subcutis of the distal limb.

Can been painful in relation to pressure and temperature.

77
Q

Aquired vascular lesions

What is this?

A

Pyogenic Granuloma

  • Most commonly affect the gingiva, fingers, lips and face.
  • Younger people
  • Gingival lesions - present during pregnancy.
78
Q

If you see a pyogenic granuloma, what should you always do?

A

Send away a specimen for histology

(They can look like amelanotic melanomas)

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