Pachydermoperiostosis associated with Glomerulonephritis: A Case Report

Abstract

Hypertrophic osteoarthropathy (HOA) is characterized by clubbing fingers and periostosis of the long bones, which can be classified as the primary or secondary form. Pachydermoperiostosis (PDP), the primary idiopathic form of HOA, is featured by pachydermia, clubbing fi ngers, and periostosis of long bones, but lack of signs of cardiopulmonary, hepatic or intestinal diseases. Other manifestations include arthritis, seborrhea, folliculitis, cutis verticis gyrata, hypertrophic gastropathy and acroosteolysis. Here we report a 43 year-old male who had clubbing of the terminal phalanges, thickening of leg and forearm skin, progressive coarsening of facial features and seborrhea for more than a decade, came to our clinic due to both knee arthritis and pitting edema of lower limbs for about one year. The histology of skin and extremity x-ray findings were also consistent with the diagnosis of PDP. In addition, owing to heavy proteinuria, renal biopsy was performed and showed mesangial proliferative glomerulonephritis. He also had a myxoma over the right forearm. Described to date, there was no report of PDP associated with glomerulonephritis and soft tissue myxoma in the literature. Because similar pathogenic mechanisms involved in the changes of skin, bone and kidney have been revealed, we suggest that the coexistence of PDP and glomerulonephritis can be pathogenically rather than coincidentally related.