A 53 y.o. with a hx of alcoholism, gout, and seizures presents with a rash.
What could it be?
Hint: he also has marked proteinuria
Our patient had an IgA related leucocytoclastic vasculitis with renal involvement and nephrotic syndrome. In children, this rash is often post viral( because IgA is the dominant antibody of immunity) and presents with a similar rash: Henoch-Schonlein purpura. In adults, cirrhosis can cause an IgA associated nephropathy. It is a common condition associated with cirrhosis and portal hypertension but does not often present with nephrotic range proteinuria.
Cirrhosis results in impaired hepatic clearance of circulating IgA immune complexes with subsequent deposition in glomeruli. Nephritic syndrome can develop with RBCs and protein in the urine
IgA nephropathy is the most common glomerulonephritis in the world with 1.3% of the population affected. It is commonest in southern Europe ,Asia and among native Americans.
Henoch Schonlein purpura in children tends to start in dependent areas.
CAUSES OF IgA INCREASE
Viral Illness with Immunoglobin vasculitis (Henoch-Schonlein purpura, AIDS, hep C, hep B
Bacterial illness- post strep, GC, syphilis
Cirrhosis
IgA myeloma
Immune diseases- lupus, rheumatoid arthritis. Wegener’s, crohn’s, celiac disease
Drugs
IgA deposition ion kidney biopsy
EVALUATION
The rash for a vasculitis is described as palpable purpura. This means the blood has extravasated into the tissue and does not blanch with pressure. The evaluation for vasculitis in our patient included a postitive ANA at 1:640 with negative double stranded DNA, hep C hep B RPR ANCA and cryoglobulins. Infectious causes like bartonella and bacterial infections were ruled out .
Initially, the rash was felt to be secondary to the naproxyn which the patient had been taking for gout. In spite of discontinuing naproxyn, the renal disease persisted and was thought to be due to his cirrhosis. The rash was treated with triamcinolone 0.1% and the renal disease was treated with Lisinopril.(ACE inhibitors lower portal pressure). Since IgA nephropathy is self-limited in 90% of cases, the treatment is based on the results of kidney biopsy. If crescents are present, indicating more active disease, steroids or immunosuppression is indicated. Our patient was managed only with Lisinopril. He returned to the ED since discharge with an alcohol withdrawal seizure. At that time his rash was gone and his kidney function was normal.
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