22.2 Angiology: Arterial Disease, Raynaud Phenomenon, Livedo, Thrombosis, Lymphoedema & Lipoedema

22.2 Angiology: Arterial Disease, Raynaud Phenomenon, Livedo, Thrombosis, Lymphoedema & Lipoedema

Peripheral Arterial Disease (PAD)

Atherosclerotic PAD shares risk factors with coronary disease: smoking, diabetes, hypertension, dyslipidaemia, age, and chronic kidney disease.

• The ankle-brachial pressure index is covered in the phlebology section. In diabetes and chronic kidney disease, calcified arteries can give a falsely high ABPI (over about 1.3), so toe pressures or transcutaneous oxygen measurements are used.
• Arterial ulcers are painful, punched out, and on the toes, heels, or shins, with cold, pale, hairless skin and absent pulses.
• Management: stop smoking, antiplatelet therapy, statins, blood pressure and diabetes control, supervised exercise, and revascularisation for limb-threatening ischaemia. High compression is contraindicated in significant arterial disease.

Raynaud Phenomenon

Episodic, cold- or stress-induced vasospasm of the digits, with a colour sequence of white (ischaemia), blue (cyanosis), and red (reperfusion).

Capillaroscopy in systemic sclerosis shows early, active, and late patterns: giant capillaries and microhaemorrhages, then capillary loss and disorganised new vessels.

Treatment:

1. Keep the whole body warm, wear gloves, and stop smoking; avoid triggering drugs.
2. Dihydropyridine calcium channel blockers (nifedipine, amlodipine) are first-line drugs.
3. Alternatives and additions: PDE5 inhibitors (sildenafil), topical nitrates, and angiotensin receptor blockers.
4. Severe attacks and digital ulcers: IV iloprost. Bosentan reduces new digital ulcers in systemic sclerosis. Botulinum toxin injections and digital sympathectomy are used in refractory cases.

Livedo

Livedoid Vasculopathy

• A non-inflammatory thrombo-occlusive disease of small dermal vessels, often with a hypercoagulable state.
• Clinical: very painful purpuric macules and small ulcers around the ankles and feet, often worse in summer, healing as porcelain-white stellate scars with telangiectasia (atrophie blanche) and livedo.
• Histology: fibrin in the walls and lumen of dermal vessels, without true vasculitis.
• Work-up: thrombophilia screen, including antiphospholipid antibodies.
• Treatment: anticoagulation, for example rivaroxaban (supported by a German trial), antiplatelet drugs, pentoxifylline, and IVIG in refractory disease, with pain control and compression if arterial flow allows.

Superficial Vein Thrombosis (Superficial Thrombophlebitis)

• A tender red cord along a superficial vein, often a varicose vein.
• Duplex ultrasound is advised, because deep vein thrombosis coexists in about a quarter of patients.
• Treatment: a thrombosis at least 5 cm long and more than 3 cm from the saphenofemoral junction is treated with fondaparinux 2.5 mg daily for 45 days (CALISTO trial), plus analgesia and compression. A thrombus within 3 cm of the junction is treated as a deep vein thrombosis.
• Recurrent or migratory thrombophlebitis without varicose veins should prompt a search for cancer (Trousseau syndrome), thrombophilia, Behçet disease, and thromboangiitis obliterans (Buerger disease), which affects young male smokers with distal ischaemia and needs complete smoking cessation. Mondor disease is thrombophlebitis of veins on the chest wall or penis.

Deep Vein Thrombosis (DVT)

• Diagnosis: clinical probability (Wells score), D-dimer, and compression ultrasound.
• Treatment: direct oral anticoagulants (rivaroxaban, apixaban) or low-molecular-weight heparin for at least 3 months. Compression relieves symptoms.
• Post-thrombotic syndrome: chronic leg pain, oedema, pigmentation, and ulceration after DVT, leading to chronic venous insufficiency.

Thrombophilia and the Skin

• Inherited: factor V Leiden (the most common inherited thrombophilia in Europeans), prothrombin G20210A, and deficiencies of protein C, protein S, and antithrombin.
• Acquired: antiphospholipid syndrome (lupus anticoagulant, anticardiolipin, or anti-beta-2-glycoprotein I antibodies, persistent for at least 12 weeks), cancer, pregnancy, and oestrogens.
• Skin signs: livedo racemosa, skin necrosis and ulcers, splinter haemorrhages, and warfarin-induced skin necrosis (painful necrosis of fatty areas in the first days of warfarin, especially in protein C deficiency). Homozygous protein C deficiency causes neonatal purpura fulminans.

Erythromelalgia and Other Acral Disorders

• Erythromelalgia: episodes of burning pain, redness, and heat of the feet or hands, triggered by warmth and relieved by cooling. Primary forms are caused by gain-of-function SCN9A mutations (sodium channel Nav1.7). Secondary erythromelalgia is linked to myeloproliferative neoplasms (essential thrombocythaemia, polycythaemia vera), where it responds to aspirin. Patients should avoid soaking in ice water, which can cause ulceration.
• Acrocyanosis: persistent painless blue-red discolouration of the hands and feet, worse in cold.
• Cholesterol embolisation: livedo racemosa, "blue toes", and ulcers after arterial catheterisation or anticoagulation, with eosinophilia and kidney injury.

Lymphoedema

• Stemmer sign: inability to pinch a fold of skin at the base of the second toe (or middle finger) indicates lymphoedema.
• International Society of Lymphology stages: 0 latent (subclinical); I pitting oedema that improves with elevation; II oedema that does not resolve with elevation, with fibrosis; III lymphostatic elephantiasis with skin changes such as papillomatosis and hyperkeratosis (elephantiasis nostras verrucosa).
• Investigations: lymphoscintigraphy or indocyanine green lymphography when the diagnosis is unclear.
• Management: complex decongestive therapy (manual lymphatic drainage, multilayer short-stretch bandaging, exercise, and skin care), then flat-knitted compression garments. Prevent cellulitis with skin care and prophylactic penicillin after repeated episodes. Surgical options include lymphovenous anastomosis, lymph node transfer, and liposuction for fat-dominant disease.
• Complications: recurrent cellulitis and, rarely, lymphangiosarcoma (Stewart-Treves syndrome) in long-standing lymphoedema.

Lipoedema

• Affects women, usually starting around puberty, pregnancy, or menopause.
• Symmetric excess fat of the legs (and often arms) that spares the feet and hands, giving a "cuff" at the ankles.
• Tender, painful, and easily bruised; Stemmer sign is negative unless secondary lymphoedema develops.
• Does not respond well to dieting. Management includes compression, exercise, weight management, psychological support, and liposuction in selected patients.