Abstract

Schoenlein Henoch Purpura (SHP) may show a cohort of symptoms such as: a palpable purpuric rash where lesions may be prominent mostly on lower extremities, abdominal pain, arthritis and nephritis. It is an inflammatory disorder characterized by an IgA-mediated vasculitis with immune complexes deposition in smaller veins, capillaries and arterioles which often occur as a response to infections (mycoplasma, streptococcus group A, Campylobacter enteritis, Helycobacter, Herpes virus, Parvovirus B19, Epstein-Barr virus), or to an exposure to allergens, drugs, some particular food, cold, or even insect bites. Symptoms are often dramatic at the onset, and the exitus occurs sadly between 30 – 50% of the patients, despite the therapeutic support, for this reason the diagnosis and the therapy should be precocious. Clinically we can observe multi organ failure, hypotension, flushing, capillary leak haemorrhage secondary to thrombocytopenia, platelet dysfunction, disseminated intravascular coagulation, shock. We have studied a young boy (19 years old) with an urticarial wheals, erythematous maculo-papules, purple spots, and larger petechiae, migrant arthritis and arthralgia, renal disease (low grade hematuria with proteinuria), weakness, necrotic and hemorrhagic plaques, with haematemesis, diarrhoea, abdominal pain, fever (38.5°/39.5° C), and increased blood level of human Parvovirus-B19 NS1-gene component. We performed a skin biopsy of SHP that showed: leukocytoclastic vasculitis, with angiocentric neutrophilic infiltrate, fibrin deposition and focal fibrinoid necrosis. We also evaluated a kidney biopsy which showed: mild increase of mesangial cellularity, with segmental capillary luminal leukocytes and scattered capillary wall fuchsinophilic deposit, while the diagnostic finding is constituted by granular mesangial IgA with C3. The management is based on support therapy, with plenty of haemotrasfusions, and immunosuppressive therapy (prednisone 40 mg os/die, and cyclophosphamide100–200 mg/daily/os for 30–60 days (may decrease inflammation, capillary permeability and suppressing granulocytes activity and migration. The prognosis is usually excellent when the syndrome is self-limited and resolves spontaneously often 4/8 weeks without recurrence. Sometimes the diagnostic investigation may show an elevated level of vascular IgA deposition (direct immunofluorescence), elevated plasma levels of C3 and C4, elevated levels of IgA, ANCA (antineutrophil cytoplasmic antibodies: pANCA anti-MPO, cANCA anti-PR3), CRP, fibrinogen, ICAM-1, and increase of TNF, IL-1, IL-6, TGF-beta that reflect endothelial cell damage and dysfunction.

Author notes

Disclosure: No relevant conflicts of interest to declare.