Concept:
Henoch-Schönlein purpura (HSP), also recognized as IgA vasculitis, is the most common systemic small-vessel vasculitis in childhood.
It is characterized pathologically by leukocytoclastic vasculitis with predominant IgA-dominant immune complex deposition in small vessels of the skin, gastrointestinal tract, joints, and kidneys.
Explanation:
• The EULAR/PRINTO/PRES consensus criteria require the presence of palpable purpura (mandatory criterion, predominantly on dependent areas such as buttocks and lower extremities) without thrombocytopenia or coagulopathy, PLUS at least one of the following:
1. Diffuse colicky abdominal pain (often complicated by intussusception)
2. Arthritis or arthralgia (acute onset, non-migratory, non-deforming)
3. Renal involvement (hematuria and/or proteinuria)
4. Biopsy showing predominant IgA deposition
• The provided immunofluorescence image typically demonstrates prominent mesangial or capillary wall IgA deposition within the renal glomerulus or dermal venules.
• Hemolytic Uremic Syndrome (HUS) is characterized by the classic triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, usually following bloody diarrhea without palpable purpura.
• Dengue fever presents with acute febrile illness, retro-orbital pain, severe myalgia, thrombocytopenia, and petechial (non-palpable) rash.
Final Answer:
The classic tetrad of palpable purpura, colicky abdominal pain, arthralgia, and renal involvement (microscopic hematuria) is diagnostic of Henoch-Schönlein Purpura (IgA Vasculitis).