A 20-Year-Old Man With a Dark, Burning Rash

Christian Beebe, MD, MBA

Disclosures

August 13, 2015

The patient meets the histopathologic criteria for solitary IgA nephropathy; the subsequent renal biopsy showed diffuse proliferative endocapillary glomerulonephritis with increased mesangial matrix and cellularity, capillary wall thickening, and lobular accentuation on light and electron microscopy with strong (3+) mesangial staining for IgA and fibrinogen, as well as kappa and lambda light chains (2+ to 3+) on immunofluorescence microscopy. However, he exhibited cardinal clinical features of Henoch-Schönlein purpura at presentation, because the skin disease is highly uncommon with IgA nephropathy alone.

The patient denied having had blood in his urine on prior urinalyses or a history of gross blood in the urine, lessening the possibility of IgA nephropathy. After further inquiry, it was confirmed that the patient had no family history of renal disease, and as such, he was unlikely to have familial IgA nephropathy.

Polyarteritis nodosa (PAN) could be considered in the differential diagnosis, but this patient displayed none of the classic signs or symptoms of fever, abdominal pain, nausea, and weight loss. Also common to PAN is the involvement of small- and medium-sized arteries, as well as subcutaneous nodules (not palpable purpura). Joint pain, however, is very common in PAN. PAN may be associated with hepatitis B or hepatitis C, but this patient's hepatitis panel was negative.

A hemorrhagic diathesis was quickly ruled out with the normal coagulation studies. Lyme disease, in contrast to Henoch-Schönlein purpura, more commonly presents with the typical target lesions of erythema migrans and a positive Lyme titer (and not with a purpuric rash). This patient had a negative Lyme antibody examination, although this finding alone does not exclude the possibility of the disease. Multiple arthralgias are more common in Lyme disease, and they seldom present with abdominal pain.

Hemolytic-uremic syndrome should be considered in the differential diagnosis, but it can be easily recognized by the presence of microangiopathic anemia. It is distinguished from Henoch-Schönlein purpura by the absence of purpura and arthralgias, and a characteristic kidney biopsy finding of a thrombotic microangiopathic lesion.

Henoch-Schönlein purpura is an autoimmune, self-limited IgA-mediated vasculitis that most commonly presents in children > 5 years. In fact, 95% of cases present in children < 10 years.[9] It has a 2:1 distribution between males and females.[10] The overall incidence is 0.14 cases per 1000 population in children aged 2-14 years.[10] This patient is in a relatively uncommon demographic stratum for the disease.

The most common signs and symptoms of the disease are palpable purpura (100%), abdominal pain (63%), gastrointestinal bleeding (33%), arthralgia (82%), nephritis (40%), and hematuria.[11] Risk factors associated with a poor prognosis for renal survival include a clinical nephrotic syndrome, decreased factor XIII activity, hypertension, and renal failure at onset.[9] It is known that severe Henoch-Schönlein purpura-associated nephritis or its progression to nephrotic syndrome is associated with a poorer prognosis.[9]

The mainstays of treatment for Henoch-Schönlein purpura are observation, intravenous hydration, and steroid therapy, mostly for the bowel symptoms. Most cases are self-limited and resolve within 4 weeks.[12,13] Cyclophosphamide is usually reserved for severe, systemic forms of vasculitis, and appropriate consultation with a nephrologist is recommended.[14]

Chronic kidney disease and end-stage renal disease are known complications of Henoch-Schönlein purpura, with incidence rates of 5% and 1.5%, respectively.[12] Corticosteroids should be instituted only after consultation with a nephrologist, because they are of questionable benefit for the kidney component.[13,15] In fact, only one randomized controlled clinical trial has been performed evaluating the use of corticosteroids as renoprotective agents in Henoch-Schönlein purpura: Huber and colleagues[15] found that early initiation of prednisone therapy did not prevent chronic kidney disease at 1 year. However, a case report described successful treatment of an adult patient with crescentic glomerulonephritis (from Henoch-Schönlein purpura) with nine rounds of double-filtration plasmapheresis and concomitant plasma replacement.[16]

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