Sch??nlein-Henoch Purpura in Children and Adults

Abstract

Schönlein-Henoch syndrome is a form of systemic small-vessel vasculitis, characterised by vascular and/or mesangial immunoglobulin A1 deposits. The main clinical manifestations are vascular purpura, predominating on the lower limbs, and articular, gastrointestinal and renal symptoms. Pulmonary, cardiac, genital and neurological symptoms have also be observed. The syndrome usually affects children, whereas it is rare in adults. The frequency of renal involvement varies between individual studies (from 20 to 100%). Renal manifestations are usually mild and transient, although chronic nephropathies may occur. Overall, an estimated 2% of children with Schönlein-Henoch purpura progress to renal failure and up to 20% of children with nephritis treated in specialised centres require haemodialysis. The renal prognosis appears to be worse in adults. Aetiological investigations are required, as a triggering factor is found in approximately half the patients (e.g. viral, bacterial and parasitic infections, drugs, toxins, systemic diseases and cancer). Dapsone has beneficial effects on cutaneous, gastrointestinal and articular manifestations in adults, especially those with chronic forms. Corticosteroids may be useful for refractory abdominal pain. Methylprednisolone pulse therapy, immunosuppressive drugs (e.g. cyclophosphamide and azathioprine), plasma exchange and polyclonal immunoglobulin therapy are beneficial in very rare life-threatening forms of the disease and in rare instances where renal function is compromised.

Petechia of the Penis: Sexual Habits or Adverse Drug Reaction?

“…Although these lesions are mostly found in other skin areas, petechia occasionally occur in the genital district under different conditions, such as lichen sclerosus or Henoch-Schönlein purpura [3,4]. Particularly, the most frequent sex practices-related petechia are those observed in oral cavity after oral sex (fellatio) [2,5].…”

Cutaneous Vasculitis with Renal Impairment in Octogenarian

“…The prognosis is usually favorable, but some patients with nephritis may progress to renal failure [2], characterized by systemic arterial hypertension, impaired renal function and glomerulonephritis [17]. polyarthralgia usually affects associated knees and ankles with spontaneous resolution [18] [19]. Cutaneous, gastrointestinal, renal and articular involvement are common features of the pathology, whereas orchitis, pulmonary haemorrhage and vasculitis of the central nervous system (CNS) are rarely evidenced [2].…”

“…In the histopathological examination, there is a cutaneous component with lesions consisting of subepidermal hemorrhages and necrotizing vasculitis of the small vessels of the dermis [19]. The deposition of IgA, a prominent feature of fluorescence microscopy [3] [20], is present in these vessels.…”

“…The pathophysiology of cutaneous vasculitis may be due to five major mechanisms: hypersensitivity mechanisms; mediated by type 1 reaction of Gel and Coombs, with active participation of eosinophils; Type 2 reaction of Gel and Coombs (antibody-mediated cytotoxicity); Immunocomplex mediated disease; and direct affection of the vessel [19].…”

“…Regarding treatment, joint pain and painful soft tissue edema usually respond to acetaminophen or non-steroidal anti-inflammatory drugs [18], but oral prednisone, from 1 to 2 mg/kg per day, may be necessary to accelerate its resolution [19]. Anti-inflammatory agents should be avoided in patients with extensive renal involvement.…”