Diagnosing the SAPHO syndrome: a report of three cases and review of literature
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CASE BASED REVIEW
Diagnosing the SAPHO syndrome: a report of three cases and review of literature Bijit Kumar Kundu & Ananta Kumar Naik & Shrinath Bhargava & Dinesh Srivastava
Received: 18 February 2013 / Revised: 27 March 2013 / Accepted: 27 March 2013 / Published online: 19 April 2013 # Clinical Rheumatology 2013
Abstract SAPHO, an acronym for synovitis, acne, pustulosis, hyperostosis and osteitis, is a heterogeneous entity with myriad presentations and features overlapping with other entities. It is a differential in patients presenting with skin and bone symptoms, either singly or in combination. Often misdiagnosed radiologically as a malignancy or infection, the diagnosis is seldom thought of. We present three cases referred to us for evaluation of findings unrelated to the presenting symptoms. After evaluation, a 99Tc bone scan was ordered, which showed the ‘bull’s head sign’ in all the three cases, confirming the diagnosis. We review the literature for SAPHO. It has a few features which point to its diagnosis and can help us to distinguish it from other seronegative arthritis. The clinician should be aware of this entity and should not hesitate to order a 99Tc bone scan. We conclude that SAPHO is not rare, but rather, it is underdiagnosed. High index of suspicion is necessary for diagnosis. A 99Tc bone scan is diagnostic and should be ordered in patients having any of the presenting features of the syndrome. We put forward the suggestion of
B. K. Kundu (*) Rheumatology Clinic, Department of Medicine, PGIMER, Dr RML Hospital, New Delhi 110001, India e-mail: [email protected] A. K. Naik Department of Orthopaedics, PGIMER, Dr RML Hospital, New Delhi 110001, India e-mail: [email protected] S. Bhargava Department of Dermatology, PGIMER, Dr RML Hospital, New Delhi 110001, India e-mail: [email protected] D. Srivastava Department of Medicine, PGIMER, Dr RML Hospital, New Delhi 110001, India e-mail: [email protected]
using 99Tc bone scintigraphy to define a ‘pre-MRI’ stage of ankylosing spondylitis. Keywords Bull’s head sign . HLA B27 . Sacroiliitis . SAPHO . 99Tc bone scan
Introduction SAPHO is an acronym for synovitis, acne, pustulosis, hyperostosis and osteitis. Its manifestations primarily affect the skin and the bones, for which it is called a skin and bone (SKIBO) disease. However, all manifestations may not be present concurrently and hence may not be correlated by even astute clinicians. Besides, it has many features, both clinical and radiological, which overlap with other well-defined disease entities, thus causing confusion in the diagnosis. The diagnostic criteria for this entity was proposed by Kahn and Khan in 1994 as multifocal osteitis with/without skin symptoms, sterile acute/chronic joint inflammation with either pustules/psoriasis of palms/soles, or acne or hidradenitis, and sterile osteitis and any one of the above skin manifestations, with any one of the criteria being sufficient for the diagnosis [1]. Bone scans of these patients are usually interpreted by the radiologist as
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