A RARE CASE OF PRIMARY HYPERTROPHIC OSTEOARTHROPATHY : CASE REPORT

Abstract

BACKGROUND: Hypertrophic osteoarthropathy (HOA) is characterized by abnormal growth of skin and osseous tissue at distal parts of the extremities, also known as orphan syndrome and Pierre Marrie-Bamberger syndrome. The peculiar bulbus deformity of the digital tips known as 2 “clubbing”, periosteal proliferation of long bones and painful effusion of synovium. HOA can be primary(idiopathic) and secondary. Although HOA is rare, secondary HOA is more common in certain diseases like chronic pulmonary disease, congenital heart disease, gastrointestinal disease, 5 and malignancies which may improve with treatment of the underlying disease. On the other hand primary HOA is idiopathic and its pathophysiology remains unclear. When idiopathic HOA is associated with pachyderma (thickened skin) is known as pachydermoperiostosis. In 4 extremely rare instance idiopathic HOA may occur without pachyderma. CLINICAL DESCRIPTION: We report a case of 4 years old female child came with complaints of pain and swelling in bilateral knee and ankle joints since 2 years, and developed pain and swelling in elbow and wrist joint since 1 year. On general examination she has grade 4 clubbing in ngers and toes. Suspecting HOA, relevant investigations sent to rule out secondary causes of HOA. After ruling out all causes of secondary HOA, we arrive at diagnosis of primary (idiopathic) HOA. MANAGEMENT: As there is no denitive management for primary HOA, we managed our patient symptomatically with painkillers. CONCLUSION: Reporting a rare case of primary HOA without pachyderma treated symptomatically with NSAIDS.