Case Report: A Patient Presents with Rare, Fulminant SAPHO Syndrome

Next, multifocal areas of vertebral bone marrow edema consistent with osteitis appear on MRI (see Figures 2A and 2B). Vertebral lesions are classically described as corner lesions owing to the bone edema usually associated with erosion found at the anterior corners of the vertebral bodies (see Figure 2B). These lesions are difficult to differentiate from infectious and malignant bone lesions, so biopsy is usually required to evaluate the differential diagnoses.

Hyperostosis noted on plain X-ray can appear and is most commonly noted in the clavicle (although the radiographs of our patient revealed hyperostosis of the proximal humerus and distal acromion). Finally, in contrast to hyperostosis lesions, osteolytic lesions may be discovered as well.^6,10

Our patient’s presentation was unique because it included all the typical imaging findings of SAPHO, and this constellation of radiographic findings was key in clinching the diagnosis.

Conclusion

SAPHO syndrome remains a poorly understood disease and is thought, at this time, to be rare. The literature describing SAPHO suggests it can present in a myriad of ways. Our case was unique because it occurred in a black man and featured fulminant onset, all the classic radiographic findings and a rarely associated dissecting cellulitis dermatosis.

Several treatment options are available for SAPHO. Our patient’s fulminant course was successfully treated with anti-TNF therapy. Physical therapy, topical steroids and antibiotics for skin lesions add to the therapeutic armamentarium.

Still, we need larger cohorts and collaborative studies to better define efficacy and outcomes in the management of SAPHO syndrome.

Ross J. Thibodaux, MD, is a rheumatology fellow in the Division of Rheumatology at the Louisiana State University Health Science Center in New Orleans.

Nirupa J. Patel, MD, is an associate professor in the Division of Rheumatology at the Louisiana State University Health Science Center in New Orleans.

References

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