Successful treatment of pyoderma gangrenosum with concomitant immunoglobulin A nephropathy: A case report and review of literature

Abstract

Pyoderma gangrenosum (PG) is an uncommon ulcerative cutaneous condition of an unknown etiology and is often associated with immune diseases. However, PG rarely shows visceral involvement, especially in the kidney. A 20-year-old female presented with pedal edema and skin ulceration of both lower limbs. The skin lesion began as an erythematous plaque and then became a blister. She also complained of abdominal distension and a decreasing urine volume. Laboratory data showed high proteinuria, hypoalbuminemia and hyperlipidemia. Her skin and kidney were biopsied. The pathological results indicated PG and immunoglobulin A (IgA) nephropathy. The patient was finally cured with prednisolone in combination with cyclosporine A (CsA).

Keywords: Immunoglobulin A nephropathy; Pyoderma gangrenosum; Treatment.

Figure 1

The right lower leg exhibited ulcerated lesions with erythematous-violaceous excavated borders and a necrotic center.

Figure 2

Light microscopy of the primary skin lesion. Massive small lymphocytes (black arrow) are arranged around blood vessels throughout the dermis [hematoxylin and eosin (HE) staining, × 200]

Figure 3

Light microscopy of the biopsied kidney tissue. The mesangial area is moderately enlarged due to an increase in mesangial cells (black arrow) and the matrix. Endothelial cells (green arrow) show diffuse proliferation and degeneration. Infiltrated neutrophils (red arrow) are present [hematoxylin and eosin (HE) staining, × 200].

Figure 4

Immunofluorescence staining of the biopsied kidney tissues. IgA showed strong positivity within the mesangium (× 200). IgA: Immunoglobulin A.

Figure 5

The skin lesions on the right lower leg were healed after one year, with only pigmentation remaining.