Purpura and microvascular occlusion

Question 1

When does warfarin necrosis usually occur?

Answer 1

3-5 days post commencing warfarin

Question 2

5 types of PPD

Answer 2

Shamberg’s disease
Itching purpura (eczematid like purpura of doucas and kapetanakis)
Pigmented purpuric lichenoid dermatoses of Gougerot and Blum
Lichen aureus
Pupura annularis telangiectoides (Majocchi’s)

Question 3

Atrophie blanche associated with

Answer 3

Associated with chronic venous hypertension and varicosities but this form does not have surrounding livedo reticularis, also seen in Antiphospholipid ab related syndromes, sickle cell ulcers.

Question 4

2 classic triads of cholesterol embolic

Answer 4

1: leg/foot pain + livedo reticularis + preservation of good peripheral pulses
2: livedo reticularis, renal insufficiency and eosinophilia.

Question 5

Achenbach’s syndrome (paroxysmal finger haematoma)

Answer 5

Recurrent painful bruising on palms & palmar aspects of fingers, likely venous rupture.

Question 6

What to exclude if you suspect PPD

Answer 6

MF
Drug causes
Infections eg beta haemolytic strep

Question 7

Who gets ppd of gougerot and Blum?

Answer 7

Men between 40-60.

Question 8

Degos disease aka

Answer 8

Malignant atrophic papulosis

Question 9

Classification of purpura

Answer 9

1) petechiae
2) macular erythema
3) ecchymoses
4) palpable pupura
5) inflammatory retiform
6) non inflammatory retiform

Question 10

Histology of PPD

Answer 10

perivascular infiltrate of lymphocytes and macrophages is centered on the superficial small blood vessels of the skin. Signs of endothelial cell swelling and narrowing of lumina may be seen. RBC extravasation and haemosiderin. Perl’s to confirm haemosiderin and Fontana-Masson to exclude melanin

Question 11

tx of Sneddons

Answer 11

No effective treatment.
Avoid smoking, OCP
Treat HTN, hyperlipidaemia
Antiplatelets in longer term, thrombolytic and vasodilators
If antiphospholipid abs or lupus anticoagulants: warfarin.

Question 12

Important hx factors in retiform pupura

Answer 12

Sx of infection, exacerbation in cold
Hx of thrombotic disease, renal disease, haematological disease, CV disease, connective tissue disease
Fam hx of thrombotic disease
Drug hx eg warfarin, levamisole in cocaine.
Miscarriage hx

Question 13

Red flags for purpura

Answer 13

• Larger or variably sized esp when no in sun damaged skin
• Numerous or widespread lesions
• Lesions occurring in crops
• Palpable lesions
• reticulate
• Associated features eg pustules, necrosis, nodules, splinter haemorrhages
• Evidence of bleeding from other sites eg haematuria
• Associated general sx

Question 14

Investigation of suspected occlusive syndromes

Answer 14

FBC, coags, elfts, ancas, biopsy

Question 15

Causes of retiform pupura

Answer 15

Non-inflammatory: microvascular occlusion, protein C and S deficiency, cholesterol emboli
Inflammatory occlusion:
-IgA vasculitis
-mixed cryo, rheumatic, PAN, microscopic polyangiitis, wegeners, churg-strauss), livedoid vasculopathy, septic vasculitis, pernio, PG.
-Calciphylaxis, heparin or warfarin necrosis, antiphospholipid ab syndrome

Question 16

Ix of retiform pupura

Answer 16

Skin biopsy – incisional : centre (rook) and edge. If infection suspected: culture
FBC, film, elfts, hep B,C
Coags, ANCAs, serum EPP, RF, ANA, C3, C4, factor V leidin, Protein C/S
Cardiolipin abs, beta2 glycoprotein I antibodies, lupus anticoagulant.
If acral/cold: cryoglobulins, cryofibrinogen

Question 17

Causes of palpable pupura

Answer 17

• LCV, urticarial vasculitis
• small and medium vessels (mixed cryoglobulinaemia, rheumatic)
• ANCA associated (Wegeners, microscopic polyangiits, churg-strauss), EED, Sweets
• EM, PLEVA, PPD, hypergammaglobulinaemic pupura of waldenstrom

Question 18

What is the Rumpel-Leede sign

Answer 18

Capillary leakage post BP cuff when pressure released. Can be checked by the Hess test (inflate to 80mmHg for 5 min), count petechiae just below the antecubital fossa. >5 is abnormal. May occur in ehlers dnalos (IV), some platelet dysfunction ,HHT

Question 19

Who gets shamberg’s?

Answer 19

Mainly young adult males

Question 20

Generalised livedo racemosa or livedo reticularis with CV lesions that cause focal neurological symptoms or signs. Associated Raynauds or acrocyanosis
F>M, typically in 4th-5th decades of life

Answer 20

sneddons

Question 21

Acroangiodermatitis (of Mali), pseudo-kaposi’s sarcoma

Answer 21

Associated with venous insufficiency or with vascular anomalies eg klippel-trenaunay. M>F
Lower legs, dorsum feet and toes.
Due to abnormal vasculature.

Question 22

Who gets itching ppd?

Answer 22

Usually adult men

Question 23

pupura at friction sites - think of…

Answer 23

Clothing contact, capillaritis,HSP, Ehlers-danlos

Question 24

Causes of neonatal pupura

Answer 24

• Deficiency of clotting factors/coagulation
• Thrombocytopenia
• Blueberry muffin
• TORCH
• Wiskott-aldrich
• Maternal ab-mediated.
• Associated with haemangioma

Question 25

Who gets majocchi's ppd?

Answer 25

Adolescents and young adults

Question 26

Ix of atrophie blanche

Answer 26

Venous and artierial Doppler | Ana, antiphospholipid abs

Question 27

Most common cutaneous findings of antiphospholipid syndrome

Answer 27

Livedo, leg ulcers, pseudovasculitis, digital gangrene, cutaneous necrosis and splinter haemorrhages.

Question 28

Causes of petechiae

Answer 28

Low platelets, dysfunctional platelets, scurvy, waldenstroms macroglobulinaemia, fixed increased pressure (eg BP cuff), valsalva

Question 29

What to think if ears are involved

Answer 29

Cryoglobuluinaemia, and other hyperviscositiy disorders | Levamisole, propylthiouracil

Question 30

Clinical and lab criteria of antiphopholipid syndrome

Answer 30

Clinical criteria: Vascular thrombosis Complications of pregnancy eg >10 weeks, premature births or 3 or more unexplained consecutive abortions before 10 weeks. Lab criteria Antiocardiolipin abs on 2 or more occasions at least 6 weeks apart. Lupus anticoagulant abs on 2 or more occasions at least 6 weeks apart Beta 2 glycoprotein on 2 or more occasions at least 6 weeks apart.

Question 31

Causes of occlusion due to platelet plugs

Answer 31

Heparin, essential thrombocytosis, polycythaemia, paroxysmal nocturnal haematuria, TTP/HUS

Question 32

Broad categories of causes of microvascular occlusion

Answer 32

``` Platelet plugs Infection cryogelling embolus coagulopathies reticulocyte occlusion Other: calciphylaxis, hydroxyurea, spider and snake bites , intravascular lymphoma ```

Question 33

Mechanisms of simple haemorrhage (5)

Answer 33

1) thrombocytopenia 2) platelet dysfunction 3) regional or localised increase in pressure 4) abnormal vascular support eg Ehlers danlos, scurvy, amyloidosis, PXE 5) capillaritis

Question 34

What causes warfarin blue toe syndrome?

Answer 34

Cholesterol emboli

Question 35

Causes of occlusion due to vessel organisms

Answer 35

ecthyma gangrenous, Lucia, vessel invading fungi, disseminated strongyloides

Question 36

What are some neoplasms/tumours to consider in the differential of pupura

Answer 36

kaposi’s sarcoma, angiosarcoma, bacillary angiomatosis

Question 37

Tx of PPD

Answer 37

Pruritis: antihistamines and topical steroids Venous stasis: compression Avoid dependency nbUVB: shamberg and lichenoid PUVA: schamberg and lichen aureus and lichenoid lichen aureus: case report of topical pimecrolimus, PUVA Case reports of griseofulvin, CsA, vit C 500mg bd + rutoside 50mg bd.

Question 38

What to think of if eyelids are involved

Answer 38

Increased venous pressure Coughing, vomiting, post endoscopy Amyloidosis (panda sign) Neuroblastoma