Research Journal of Medical Sciences
ISSN: Online 1993-6095ISSN: Print 1815-9346
Abstract
identified in 1935 and can manifest in three forms complete, incomplete and forme fruste. A 40 year old male presented with a 15 year history of progressive joint pain involving wrists, knees and ankles, along with dyspnea on exertion. He also noticed enlargement of extremities, had a history of severe anemia requiring blood transfusion and a family history of similar skeletal deformities. Physical examination revealed coarsening of facial features, skin furrowing, deep nasolabial folds and severe hyperhidrosis. Grade 4 clubbing of fingers and toes, soft tissue and bony enlargement over extremities were observed. Hemoglobin was 6.4 g dL-1 with a hypochromic microcytic smear. Radiographic findings showed symmetrical calcification along long bones, periosteal bone formation and cortical thickening. PDP was initially described in 1935 and is also known as the Touraine Solente Golé syndrome. It primarily affects males and often has a familial component. The syndrome presents with hyperhidrosis, extremity enlargement, arthritis, coarse facial features and more. The pathogenesis is not fully understood but is associated with specific genes and elevated prostaglandin E2 levels. Severe anemia and bone marrow issues have been reported in some cases, potentially due to bone encroachment on the medullary cavity. Pachydermoperiostosis is a rare syndrome with characteristic clinical and radiological features. Diagnosis can be challenging due to its rarity and overlapping symptoms with other conditions like acromegaly. Clinicians should consider PDP in patients with these unique features for accurate diagnosis and management. Pachydermoperiostosis, primary hypertrophic osteoarthropathy, case report, clubbing, hyperhidrosis, anemia.
How to cite this article:
Zubin Vaid and Dilip Karnad. Primary Hypertrophic Osteoarthropathy (Pachydermoperiostosis): Clinical Insights From a Case Report.
DOI: https://doi.org/10.36478/10.59218/makrjms.2023.7.1145.1148
URL: https://www.makhillpublications.co/view-article/1815-9346/10.59218/makrjms.2023.7.1145.1148