1. Polyarteritis nodosa
Polyarteritis nodosa (PAN) is a life-threatening systemic necrotizing segmental vasculitis
that involves vessels ranging in size from arterioles to medium-sized arteries.
Cutaneous manifestations are infrequent but may include palpable purpura (small-vessel
cutaneous vasculitis) or, less often, livedo reticularis, cutaneous nodules, ulcers, and
peripheral gangrene (medium to large vessel vasculitis).
2. Cutaneous polyarteritis nodosa
A necrotizing vasculitis affects small and medium-sized muscular wall arteries in the
deep dermis and subcutis. Major organs are not involved, but the disease is chronic and
recurrent.
What should I look for?
• Painful cutaneous nodules, palpable purpura, ulceration, and livedo reticularis on the
legs.
• Fever, malaise, arthralgia, and myalgia.
• Peripheral neuropathy.
What should I do?
• Screen for systemic vasculitis. Laboratory findings are unremarkable, except for
leucocytosis and an elevated ESR.
• Ensure the patient is not hypertensive.
• Take a deep incisional biopsy to demonstrate vascular pathology in arterioles or small
arteries.
• Control pain with NSAIDs, paracetamol, morphine, or amitriptyline.
• Try compression bandaging (may not be tolerated).
• Consider pentoxifylline or low-dose methotrexate (evidence lacking for efficacy).
3. Giant cell arteritis (temporal arteritis)
This granulomatous panarteritis of the large and medium sized arteries, particularly those
of the head and neck, generally affects patients >60 years of age. Some patients also have
polymyalgia rheumatica. Both conditions are commoner in women.
Complications include retinal artery inflammation with sudden irreversible blindness or,
less often, stroke.
What should I look for?
• A history of recent severe headache, scalp tenderness (noticed when combing hair or
lying on a pillow), jaw claudication, and/or visual disturbance (transient loss, diplopia,
ptosis).
• Systemic symptoms such as fever, malaise, or weight loss.
• Rarely, a tender non-pulsatile temporal artery or a tender scalp nodule that may
resemble a BCC, particularly if ulcerated.
• Pallor of the optic disc, retinal haemorrhages and exudates, optic atrophy (late finding).
What should I do?
• Measure ESR and CRP: usually very high.
• Arrange a biopsy of the temporal artery, but disease is patchy, and the artery may look
normal.
• Uncomplicated giant cell arteritis (no jaw or tongue claudication or visual symptoms):
prednisolone 40–60mg/day, until resolution of symptoms and laboratory abnormalities.
Visual loss or amaurosis fugax: IV methylprednisolone 500mg to 1g/day for 3 days.