An 8-year-old child has had abdominal pain and dark urine for 10 days. Physical examination shows blotchy purple skin lesions on the trunk and extremities. Urinalysis shows hematuria and proteinuria. Serologic test results are negative for MPO-ANCA (P-ANCA) and PR3-ANCA (C-ANCA). A skin biopsy specimen shows necrotizing vasculitis of small dermal vessels. A renal biopsy specimen shows immune complex deposition in glomeruli, with some IgA-rich immune complexes. Which of the following is the most likely diagnosis?
Correct Answer: Henoch-Schonlein purpura
Description: In children, Henoch-Schonlein purpura is the multisystemic counterpart of the IgA nephropathy seen in adults. The immune complexes formed with IgA produce the vasculitis that affects mainly arterioles, capillaries, and venules in the skin, gastrointestinal tract, and kidney. In older adults, giant cell arteritis is seen in external carotid branches, principally the temporal artery unilaterally. Polyarteritis nodosa is seen most often in small muscular arteries and sometimes veins, with necrosis and microaneurysm formation followed by scarring and vascular occlusion. This occurs mainly in the kidney, gastrointestinal tract, and skin of young to middle-aged adults. Takayasu arteritis is seen mainly in children and involves the aorta (particularly the arch) and branches such as coronary and renal arteries, with granulomatous inflammation, aneurysm formation, and dissection. Telangiectasias are small vascular arborizations seen on the skin or mucosal surfaces.
Category:
Pathology
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