Pachydermoperiostosis (idiopathic hypertrophic osteoarthropathy)--a case report and review.

National Skin Centre, Singapore.
Annals of the Academy of Medicine, Singapore (Impact Factor: 1.15). 12/1993; 22(6):939-42.
Source: PubMed


We report a Chinese patient with the complete form of pachydermoperiostosis. He presented with typical features consisting of palmoplantar hyperhidrosis, clubbing with spade-like enlargement of the hands and feet, cutis verticis gyrata, thickening and furrowing of the facial and forehead skin, and irregular thickening of the cortical bone with subperiosteal new bone formation involving the radii, ulnae. tibiae and fibulae. Classically, pachydermoperiostosis is characterised by a triad of finger clubbing, periosteal new bone formation, skin and soft tissue changes, giving an acromaegaloid look. Although rare, it is important to recognise this condition as misdiagnosis may subject the patient to unnecessary investigations and worry. The typical features and the self-limiting nature of this entity are emphasised.

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    ABSTRACT: Os autores relatam um caso de osteoartropatia hipertrófica do tipo primária em paciente de 29 anos de idade, com dores articulares e aumento de volume das extremidades há 15 anos. Ao exame físico apresentava baqueteamento dos dedos e unhas em "vidro de relógio". A osteoartropatia hipertrófica foi considerada como primária porque foram excluídas todas as causas da forma secundária. As principais alterações radiológicas foram: espessamento das camadas corticais dos ossos tubulares e reação periosteal contínua do tipo lamelar. Não foram notadas alterações dos espaços articulares.
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    ABSTRACT: Hypertrophic pulmonary osteoarthropathy is a paraneoplastic syndrome seen in patients with lung cancer. This condition is characterized by the presence of digital clubbing, periosteal thickening, synovial thickening, and severe pain of the affected joints. Other syndromes exhibiting clubbing may or may not have underlying diseases causing their manifestation. An example is primary hypertrophic osteoarthropathy, or pachydermoperiostosis. While clubbing makes up part of the clinical picture in both hypertrophic pulmonary osteoarthropathy and hypertrophic osteoarthropathy, the latter has no underlying disease associations. Rather, primary hypertrophic osteoarthropathy is familial, idiopathic, and has a chronic course often beginning during puberty in males. Secondary hypertrophic osteoarthropathy is an acquired form of clubbing that is classically associated with lung disease. However, it has also been associated with diseases of the heart, liver, and intestines. In the setting of pulmonary malignancy, secondary hypertrophic osteoarthropathy is known as hypertrophic pulmonary osteoarthropathy. Hypertrophic pulmonary osteoarthropathy has a distinct constellation of clinical findings that includes intractable pain often refractory to treatments other than resolution of the underlying disease process. The authors herein report a case of hypertrophic pulmonary osteoarthropathy masquerading as recurrent lower extremity cellulitis with chronic hand and foot pain in the setting of pulmonary malignancy that responded dramatically to intravenous pamidronate disodium (a bisphosphonate). Given the rarity of hypertrophic osteoarthropathy associated with lung cancer and the difficulty with pain management in such circumstances, the authors present the following case in which pain was mitigated by treatment with bisphosphonate therapy.
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