Abstract

Introduction: Pachydermoperiostosis (PDP) or primary hypertrophic osteoarthropathy (OAH) is a rare hereditary disorder. We report the case of a family with PDP in its complete form. 

Clinical case: We report the case of a 25-year-old man suffering from polyarthralgia with palmoplantar hypertrophy. Its history began in 2015 with the progressive installation of diffuse polyarthralgia more pronounced in large joints (knees, ankles, elbows) with diffuse bone pain and a history of pathological fracture of the left 3 metatarsal without trauma. The patient described hyper sudation, seborrhoea and increased thickening of the forehead folds and palmoplantar hypertrophy during the last 3 years. Skin examination noted oily skin, glistening and puffy, pachydermia of the extremities with a pleated thickening of the forehead, without ptosis. Palmoplantar hypertrophy and digital clubbing were evident with pudgy fingers and toes. The patient shod 44 against 41 three years ago. The clinical examination also found palmoplantar hyperhidrosis and free joints, with no inflammatory signs. The diagnosis of PDP was retained, and the patient was put on colchicine. We noted consanguinity of the parents and the physical examination of the other members of the family found the same manifestations in the father and the brother with variable intensity. 

Conclusion: PDP is a benign genetic affection that primarily affects the skin and bones. Its pathophysiological mechanism remains unclear. 

Keywords: Pachydermoperiostosis; Primary Hypertrophic Osteoarthropathy; Cutis Verticis Gyrate

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Citation

Citation: Thabet Maissa., et al. “Pachydermoperiostosis in its Complete Family form". Acta Scientific Medical Sciences 4.4 (2020): 56-59.

Copyright

Copyright: © 2020 Thabet Maissa., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.