Arch Hand Microsurg.  2019 Dec;24(4):351-357. 10.12790/ahm.2019.24.4.351.

A Case of SAPHO Syndrome with Hyperostosis of the Ulna

• Bae JY
• Lee SH
• Ko HC
• Park C
• Kang SW
• Kwak SH

• KMID: 2464471
• DOI: http://doi.org/10.12790/ahm.2019.24.4.351

Abstract

SAPHO syndrome is a rare disease comprising of synovitis, acne, pustulosis, hyperostosis, and osteitis and osteoarticular manifestations usually involve anterior chest wall, spine, or pelvis. Among these features, hyperostosis of long bone was rarely reported on the upper extremity. The present case is about a 49-year-old male with painful hyperostosis of his ulna, diagnosed with SAPHO syndrome. The diagnostic process and the progress after two years are described.

Keyword

SAPHO syndrome; Hyperostosis; Ulna

MeSH Terms

Acne Vulgaris
Acquired Hyperostosis Syndrome*
Humans
Hyperostosis*
Male
Middle Aged
Osteitis
Pelvis
Rare Diseases
Spine
Synovitis
Thoracic Wall
Ulna*
Upper Extremity

Figure

• 

  Fig. 1 Antero-posterior and lateral wrist radiographs show remained metal material. Three years after metal removal, the forearm radiographs show diffuse thickening and sclerosis of midshaft of the right ulna. Cortical thickening and sclerotic lesion of the ulna was enlarged two years after the bone biopsy.

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  Fig. 2 Axial T1- and fat-suppressed T2-weighted show diffuse cortical thickening and intramedullary sclerosis of midshaft of the right ulna without cortical nidus, bone marrow edema, periosteal reaction, lytic soft tissue lesion, or multiplicity. Technetium 99m-methyl diphosphonate bone scan shows intense uptake in bilateral sternoclavicular joints (bull's head sign) and right ulna.

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  Fig. 3 The specimen composed of dense, compact bone and broad sclerotic trabeculae of mature bone containing rare inflammatory cells (H&E stain, ×40).

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  Fig. 4 Multiple deep-seated vesicles were shown on both palms and soles. Onycholysis of both fingernails was found.

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  Fig. 5 Right and left oblique rib radiogrphs do not show evidence of sternoclavicular joint involvement. Axial computed tomography image shows symmetric cortical thickening and sclerosis of bilateral sternoclavicular joints.

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