Prevalence and Characteristics of Nonblanching, Palpable Skin Lesions With a Linear Pattern in Children With Henoch-Schönlein Syndrome

Abstract

inear nonblanching skin lesions sometimes develop in apparently healthy skin following mechanical friction. This tendency, often termed Köbnerization, is common in psoriasis, lichen planus, vitiligo, and many bullous dermatoses. 1 Very rarely, such lesions have also been documented in patients with Henoch-Schönlein syndrome, an immunoglobulin A-dominant leukocytoclastic small vessel vasculitis. [2][3][4][5][6][7][8] This report presents our prospective experience with linear nonblanching skin lesions in children with this vasculitis syndrome. MethodsTen years ago, one of us (M.G.B.) observed, for the first time, horizontal lesions on the waistline of a boy with Henoch-Schönlein syndrome. 3 Between January 1, 2010, and Decem-ber 31, 2015, all children with Henoch-Schönlein syndrome who presented to this pediatric hospitalist underwent a structured skin examination in addition to the usual clinical and laboratory evaluation (complete blood cell count, C-reactive protein, and urinalysis). Significant emphasis was put on the presence of nonblanching, palpable lesions with a linear pattern and a length of at least 3 cm (subsequently referred to as linear lesions) on the legs, groin, waistline, wrists, or forearms. The possible association of linear lesions with mechanical frictions was also addressed.The diagnosis of Henoch-Schönlein syndrome required the fulfillment of the Ankara 2008-EULAR/PRINTO/PRES (European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society) criteria. The cutaneous, abdominal, articular, and renal involvement was graded on a scale from 0 to 3, where 0 indicates absent, 1 indicates mild, 2 indicates mod-IMPORTANCE Linear nonblanching skin lesions are thought to occur very rarely in patients with Henoch-Schönlein syndrome.OBJECTIVE To examine the prevalence and characteristics of linear nonblanching skin lesions in children with Henoch-Schönlein syndrome.

A challenging case on diagnosing Henoch–Schönlein purpura

“…However, on day 32 after the first onset of abdominal pain, purpura appeared on the extensor surfaces of lower extremities (Figure 2(a)). No other cutaneous lesions, such as linear nonblanching lesion or bullous lesion, 11,12 were observed. Thus, nonthrombocytopenic purpura in lower extremities, together with recurrent abdominal pain, indicated the diagnosis of HSP.…”

Köbner and Pastia Signs in Acute Hemorrhagic Edema of Young Children: Systematic Literature Review

“…The Köbner sign occurs in approximately 10% of patients with a skin-limited or skin-predominant vasculitis [ 4 ]. Furthermore, this sign has been recently identified in approximately every fourth child with Henoch–Schönlein purpura, the most common childhood vasculitis [ 90 ]. Controversy exists as to whether hemorrhagic edema of young children represents the infantile variant of Henoch–Schönlein purpura or whether it is a similar but distinct entity.…”

Koebner phenomenon in leukocytoclastic vasculitis: A case report and an updated review of the literature

“…One study found that 10% of patients with LCV presented with KP (n = 98), 14 while the other found that 26% of children hospitalized with HSP presented with KP (n = 31). 15 Interestingly, there have also been two case reports on the topic of reverse KP in LCV, in which skin lesions have disappeared at the sites of trauma. 5,16…”