G Model BONSOI-4180; No. of Pages 2
ARTICLE IN PRESS Joint Bone Spine xxx (2015) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Letter to the Editor
Pachidermoperiostosis as a cause of massive joint effusion with polyarticular involvement mimicking juvenile idiopathic arthritis: A case report
a r t i c l e
i n f o
Keywords: Pachydermoperiostosis Arthritis Juvenile idiopathic arthritis Rare diseases
We describe a 19-year-old Caucasian man who was admitted for the first time to our department because of bilateral massive knee effusion and swelling of wrists, ankles, metacarpophalangeal (MCP) and interphalangeal (IFP) joints without tenderness (Fig. 1). Symptoms started four years earlier when he was diagnosed elsewhere as suffering with juvenile idiopathic arthritis and received methotrexate 7.5 mg/week for one year without clinical improvement. His past medical history was unremarkable. At admission, clinical examination revealed thick and hypoelastic skin with enlarged skinfold of the head and forehead (cutis verticis gyrata) associated with seborrhea and palmoplantar hyperhidrosis. Hands examination showed digital clubbing. He reported that his father had the same symptoms, but milder. Laboratory tests were normal. Serum rheumatoid factor, anti-CCP, ANA, ENA, ANCA and anti-dsDNA antibodies were all negative. Parathyroid hormone, serum 25-OH vitamin D levels, basal and stimulated GH levels, ACTH, cortisol, TSH, fT3, fT4, LH, FSH and testosterone were within normal levels. Fecal occult blood test and cancer biomarkers were negative. Joint radiographs revealed hypertrophic metacarpal and phalangeal bones with thickening of cortical bone and deformation of distal epiphysis of tibia and femur bilaterally (Fig. 1). Joint surfaces were normal and did not show erosive damage. Plain skull x-ray did not show enlargement of sella turcica. Magnetic resonance imaging (MRI) of both knees demonstrated huge joint effusion, mostly involving the suprapatellar recess, and synovial proliferation with contrast enhancement (Fig. 2) but no bone edema or active periostitis on STIR sequences. Whole-body bone scintigraphy using radiolabeled (99m)Tc-pertechnetate revealed increased turnover of periarticular bones. Left knee arthrocentesis was performed and about 1000 cc of a yellow synovial fluid with preserved viscosity was drained; synovial fluid analysis showed negative gram stain with
ARTICLE IN PRESS Joint Bone Spine xxx (2015) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Letter to the Editor
Pachidermoperiostosis as a cause of massive joint effusion with polyarticular involvement mimicking juvenile idiopathic arthritis: A case report
a r t i c l e
i n f o
Keywords: Pachydermoperiostosis Arthritis Juvenile idiopathic arthritis Rare diseases
We describe a 19-year-old Caucasian man who was admitted for the first time to our department because of bilateral massive knee effusion and swelling of wrists, ankles, metacarpophalangeal (MCP) and interphalangeal (IFP) joints without tenderness (Fig. 1). Symptoms started four years earlier when he was diagnosed elsewhere as suffering with juvenile idiopathic arthritis and received methotrexate 7.5 mg/week for one year without clinical improvement. His past medical history was unremarkable. At admission, clinical examination revealed thick and hypoelastic skin with enlarged skinfold of the head and forehead (cutis verticis gyrata) associated with seborrhea and palmoplantar hyperhidrosis. Hands examination showed digital clubbing. He reported that his father had the same symptoms, but milder. Laboratory tests were normal. Serum rheumatoid factor, anti-CCP, ANA, ENA, ANCA and anti-dsDNA antibodies were all negative. Parathyroid hormone, serum 25-OH vitamin D levels, basal and stimulated GH levels, ACTH, cortisol, TSH, fT3, fT4, LH, FSH and testosterone were within normal levels. Fecal occult blood test and cancer biomarkers were negative. Joint radiographs revealed hypertrophic metacarpal and phalangeal bones with thickening of cortical bone and deformation of distal epiphysis of tibia and femur bilaterally (Fig. 1). Joint surfaces were normal and did not show erosive damage. Plain skull x-ray did not show enlargement of sella turcica. Magnetic resonance imaging (MRI) of both knees demonstrated huge joint effusion, mostly involving the suprapatellar recess, and synovial proliferation with contrast enhancement (Fig. 2) but no bone edema or active periostitis on STIR sequences. Whole-body bone scintigraphy using radiolabeled (99m)Tc-pertechnetate revealed increased turnover of periarticular bones. Left knee arthrocentesis was performed and about 1000 cc of a yellow synovial fluid with preserved viscosity was drained; synovial fluid analysis showed negative gram stain with
