Cutaneous Manifestations of Small-Vessel Leukocytoclastic Vasculitides in Childhood

Abstract

In childhood, cutaneous small-vessel vasculitides include Henoch-Schönlein syndrome, a systemic vasculitis, and Finkelstein-Seidlmayer syndrome, a skin-limited vasculitis. Both Henoch-Schönlein and Finkelstein-Seidlmayer syndromes are seen more frequently in white or Asian compared with black children and occur especially in winter and spring with a male-to-female ratio of approximately 2:1. In everyday clinical practice, both conditions are diagnosed on clinical grounds without histological confirmation. The characteristic cutaneous hallmarks of Henoch-Schönlein syndrome include a purpuric rash in all and a subcutaneous edema in approximately every second case, which are often preceded by non-specific red or pink macular elements that mimic a non-itching urticarial rash. Recent data point out that Henoch-Schönlein children often present further cutaneous findings such as Köbnerization, Rumpel-Leede capillary fragility phenomenon, and blistering eruptions. Children with Finkelstein-Seidlmayer syndrome are usually ≤24 months of age and not ill-appearing. They present with (a) large, round, red to purpuric plaques (often with a targetoid appearance) predominantly over the cheeks, ears, and extremities and (b) often tender non-pitting edema of the distal extremities, ears, and face (without pruritus). Both in Henoch-Schönlein syndrome and Finkelstein-Seidlmayer syndrome, there is often scrotal involvement. The cutaneous findings remit without sequelae within 2 months in Henoch-Schönlein and 3 weeks in Finkelstein-Seidlmayer syndrome.

Immune deposits in skin vessels of patients with acute hemorrhagic edema of young children: A systematic literature review

“…Since the diagnosis of AHE is mostly made on a clinical basis, the histology and especially the presence of immune deposits have been only sporadically characterized . These results show that immune deposits in skin vessels, most frequently complement C3, are common in this condition.…”

“…In the latter condition, the histological findings include a leukocytoclastic vasculitis and deposition of immunoglobulin A in all cases. Deposition of complement C3, immunoglobulin G, or immunoglobulin M is also detected in many cases . However, these deposits sometimes disappear with time due to proteolysis and phagocytosis .…”

“…Cases published between 1970 and 2018 with both the clinical and histological diagnosis of AHE and who had testing for immue deposits in lesional biopsy were eligible for the present study. The diagnosis of AHE made in the original reports was reviewed using recognized clinical (not ill‐appearing infant; purpuric targetoid lesions predominantly over the cheeks, ears, and extremities; often tender non‐pitting edema of the face, auricles, and extremities; no pruritus or excoriations; no evidence of articular, abdominal, or renal involvement; recovery within 21 days) and histological (non‐granulomatous small‐vessel leukocytoclastic vasculitis) criteria . Patients presenting with features consistent with the diagnosis of HSP (palpable purpura predominantly on the lower extremities and buttocks associated with at least one of the three following characteristics: abdominal pain, arthritis or arthralgia, or renal involvement) were excluded …”

Altered B cells homeostasis in child-onset immunoglobulin A vasculitis

“…Immunoglobulin A vasculitis (IgAV), formerly referred to as Henoch–Schönlein purpura, is the most common form of childhood vasculitis. Skin biopsy of the vasculitis lesions reveal small-vessel leukocytoclastic vasculitis [1, 2]. The scarce epidemiological data for childhood IgAV, mainly pertaining to European populations, indicates annual incidence rates from approximately, 3 to 27 in every 100,000 children [3].…”

Rumpel‐Leede Phenomenon—An alarming rash with benign etiology in 4 healthy infants

“…In children, it has been associated with bleeding diatheses such as idiopathic thrombocytopenic purpura (ITP) and Henoch‐Schönlein purpura …”