Vasculitis is an umbrella term for various conditions which involve inflammation of the blood vessel walls and are multisystem in nature. Vasculitis as a whole topic is beyond the scope of Skin Deep, but it is important to recognise the common forms.
Vasculitis is often the cause of organ specific or systemic disease (primary), with the two most common forms of vasculitis in children being:
- Henoch-Schönlein purpura (HSP): predominantly small vessel vasculitis causing a triad of arthritis, colicky abdominal pain and palpable, papular purpuric rash. The peak age of onset is between 4 and 6 years, with males more commonly affected than females (~2:1). Most cases have a benign course with complete resolution of symptoms within 6 weeks. Symptomatic management with NSAIDS and corticosteroids for arthritis and abdominal pain may also be appropriate.
- Kawasaki disease: systemic small- and medium vessel disease with coronary arteritis leading to coronary artery aneurysms as the most important complication (20-30%). This usually presents with persistent fever, bilateral congestion of the ocular conjunctivae, strawberry tongue, erythema of palms and soles, a polymorphous exanthem and cervical lymphadenopathy. Ninety percent of cases occur in children under 5 years, with a higher incidence in east Asian ethnicities. Treatment options include high dose IV gammaglobulins and low dose aspirin, with cardiac follow-up.
Vasculitis can also be a sign of underlying disorders (secondary):
- Infections: Viral (hepatitis A/B/C, HIV, CMV), Bacterial (Neisseria, mycobacteria), Fungal (Candida, aspergillus)
- Malignancies: lymphoma, lymphoproliferative syndrome
- Autoimmune/ inflammatory conditions: systemic lupus erythematosus, juvenile dermatomyositis
- Medications or drug exposure: amphetamines, cocaine
Other forms of paediatric vasculitis which are also common in adults have been organised in the EULAR (European League against Rheumatism/ PReS (Pediatric Rheumatology European Society) classification criteria for childhood vasculitis (Table 1).
Table 1. EULAR/ PReS classification of paediatric vasculitis
|I. Predominantly large vessel||Takayasu arteritis|
|II. Predominantly medium vessel||Kawasaki disease|
Childhood polyarteritis nodosa
|III. Predominantly small vessel||(A) Granulomatous|
Wegener’s granulomatosis (Granulomatosis with polyangiitis)
Isolated cutaneous leucocytoclastic vasculitis
Hypocomplementic urticarial vasculitis
|IV. Other||Behçet disease|
Vasculitis secondary to infection, malignancy, drugs
Vasculitis associated with connective tissue disease
Isolated vasculitis of the central nervous system
More comprehensive discussion of individual forms of vasculitis, important differentials and treatment options is discussed in the following for further reading:
Also check out Tessa Davis’ DFTB analysis on the prospective multicentre cohort study of Petechiae in Children (PiC)
Full article of PiC Study: https://www.thelancet.com/journals/laninf/article/PIIS1473-3099(20)30474-6/fulltext