J Cardiovasc Ultrasound.  2009 Dec;17(4):135-137. 10.4250/jcu.2009.17.4.135.

Pachydermoperiostosis Accompanied by Heart Failure

• Shin KC
• Chung WJ
• Lee KY
• Shin MS
• Kim SH
• Jo YJ
• Park YM
• Ahn TH
• Choi IS
• Shin EK

• KMID: 1473716
• DOI: http://doi.org/10.4250/jcu.2009.17.4.135

Abstract

Pachydermoperiostosis or primary hypertrophic osteoarthropathy is an uncommon disease of acromegaloid facial feature, but characterized by unique phenotype (digital clubbing and pachydermia) and distinctive radiographic appearances like periostosis. We experienced a case with complete form of pachydermoperiostosis accompanied by heart failure. He presented with typical features consisting of clubbing with enlargement of the hand, thickening of facial skin and periosteal new bone formation involving lower leg. Echocardiography revealed severely decreased left ventricular systolic function. Treatment with medications resulted in an improvement of cardiac function and symptom. There is no previous report documenting pachydermoperiostosis accompanied by heart failure. We report that case for the first time.

Keyword

Heart failure; Systolic; Echocardiography; Primary hypertrophic osteoarthropathy

MeSH Terms

Echocardiography
Hand
Heart
Heart Failure
Leg
Osteoarthropathy, Primary Hypertrophic
Osteogenesis
Phenotype
Skin

Figure

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  Fig. 1 Photograph of the patient's face shows deep frontal folds (furrowing) and oily facial skin.

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  Fig. 2 Photograph of the patient's both hands reveals clubbing of fingers, swollen interphalangeal joints and round turtle-back-shaped nails.

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  Fig. 3 The X-ray of right lower leg demonstrates irregular outline and periosteal new bone formation of the calcaneus and talus bone (lower and middle arrows). Also, periosteal new bone formation is at the distal right tibia (upper arrow).

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  Fig. 4 Two-dimensional echocardiography on admission. Parasternal long axis view (A: end-systolic, B:end-diastolic) and apical 4 chamber view (C: end-systolic, D: end-diastolic) show eccentric left ventricular hypertrophy and enlarged left atrium.

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  Fig. 5 Two-dimentional echocardiography on 3 months later. Parasternal long axis view (A: end-systolic, B: end-diastolic) and Apical 4 chamber view (C: end-systolic, D: end-diastolic) show normalized left ventricular internal diameter compared with that on admission (Fig. 4).

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