Urticaria is marked by itchy wheals that vary in flare-type dermal reactions, distribution, number and size.1 Urticaria can be an acute (less than 6 weeks) or a chronic urticaria (more than 6 weeks).2 Chronic urticaria (CU) is either idiopathic chronic (55%) and autoimmune chronic (45%) without urticarial vasculitis
and physical urticaria.
can also mimic Schnitzler syndrome; however, with urticarial vasculitis
, skin biopsy should reveal features of true vasculitis with fibrinoid necrosis of small vessel walls, which should not be present in Schnitzler syndrome.
This patient was unique, in that she had recurrent urticaria, which is a very rare manifestation of Dm, whereas other autoimmune disorders like SLE, dermatomyositis, polymyositis, Still's disease have an association with chronic urticaria.3 A case of Dm with urticarial vasculitis
has been reported in a patient of nasopharyngeal carcinoma.4 Eye lid oedema in association with erythema and telangiectasia is a common feature of dermatomyositis.
Purpuric eruptions and urticarial vasculitis
are categorized as cutaneous vasculitis and together accounted for 42% of all skin lesions.
syndrome effectively treated with dapsone and pentoxifylline.
Other entities to consider in the differential diagnoses are urticarial vasculitis
, cryoglobulinaemic vasculitis, systemic lupus erythematosus and chronic idiopathic urticaria.
On punch (skin) biopsy, an increase in perivascular neutrophilic and eosinophilic cells was demonstrated in accordance with urticarial vasculitis
Nomenclature of Small Vessel Vasculitis (a) Immune Complex Vasculitis ANCA-Associated Vasculitis Anti-C1q vasculitis Microscopic polyangiitis Anti-GBM vasculitis Granulomatosis with Cryoglobulinemic vasculitis polyangiitis IgA vasculitis Eosinophilic granulomatosis Hypocomplementemic with polyangiitis urticarial vasculitis
Abbreviations: ANCA, antineutrophil cytoplasmic antibody;GBM, glomerular basement membrane disease;IgA, immunoglobulin A.
In the diagnostic procedure, skin biopsy plays an important role especially in excluding the diagnosis of urticarial vasculitis
with the predominant elements of leukocytoclastic vasculitis.
A case of ENL clinically presenting as urticarial vasculitis
that was then diagnosed to have LL has been reported by Funk WK et al.
, erythema multiforme and granuloma annulare were rare findings in our patients.
(UV) is a variant of leukocytoclastic vasculitis usually involving persistent hives.