Systemic lupus erythematosus presenting with normocomplementemic urticarial vasculitis in a 4‐year‐old girl

Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria

“…7,8,11,12 Fewer than 10 pediatric cases of UV have been reported in the last 20 years, with a female predominance (about two thirds) and an age varying from 9 months to 12 years; the principal associated manifestations were renal involvement (3 cases), pulmonary disease (2 cases), and systemic lupus erythematosus (SLE; 2 cases), with hypocomplementemia in the majority of cases (5 of 7 cases). [17][18][19][20][21][22][23] Although UV is a sporadic disease, a pair of identical twins with hypocomplementemic UV (HUV) has been reported. 24 UV is believed to result from the deposition of immune complexes on the blood vessel wall.…”

Pulmonary hemosiderosis with normocomplementemic urticarial vasculitis in a child

“…Urticarial vasculitis that is characterised by urticarial lesions persisting more than 24 hours and leukocytoclastic vasculitis of dermal vessels was diagnosed in this case by skin biopsy findings that demonstrated perivascular neutrophilic infiltration and fibrinoid necrosis [6]. This clinico-pathological condition may be associated with normal or decreased complement levels [6,12].…”

“…Urticarial vasculitis that is characterised by urticarial lesions persisting more than 24 hours and leukocytoclastic vasculitis of dermal vessels was diagnosed in this case by skin biopsy findings that demonstrated perivascular neutrophilic infiltration and fibrinoid necrosis [6]. This clinico-pathological condition may be associated with normal or decreased complement levels [6,12]. It is thought to be due to type 3 hypersensitivity reaction because circulating immune-complexes have been encountered in 30-75% of patients and these are thought to deposit in blood vessel walls and activated complement cascade [13].…”

“…It is thought to be due to type 3 hypersensitivity reaction because circulating immune-complexes have been encountered in 30-75% of patients and these are thought to deposit in blood vessel walls and activated complement cascade [13]. Urticarial vasculitis may be associated with connective tissue diseases especially systemic lupus erythematosus [6]. In only one case has pulmonary disease been previously reported to be associated with hypocomplementemic urticarial vasculitis [7,12,13].…”

“…However, the number of idiopathic cases (IPH) has gradually decreased by definition and diagnosis of vasculitic syndromes [1,[3][4][5]. One of the rarely reported causes is urticarial vaculitis (UV) characteriaed by persistent urticarial lesions that are associated with leukocytoclastic vasculitis of dermal vessels [4,6]. However association with PH is reported in a very few cases in literature which were reported to be hypocomplementemic type [7].…”