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A case of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome presenting with hypertrophic pachymeningitis

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Highlights

  • We report a case of SAPHO syndrome presenting with hypertrophic pachymeningitis (HP).

  • We summarize the clinical features of similar cases previously documented.

  • The present case emphasizes that SAPHO syndrome can be an unusual cause of HP.

Abstract

A 43-year-old woman with a 3-year history of headache, fever, and swelling of the forehead, presented to our hospital. A general examination revealed palmar and plantar pustules. Blood analyses showed an elevated white blood cell count, C-reactive protein level, and erythrocyte sedimentation rate. Brain MRI revealed a partially thickened cranial bone with gadolinium enhancement, and also abnormally enhanced dura mater. Bone scintigraphy showed involvement of the cranial bone and bilateral sternoclavicular joints. Palmar skin biopsy indicated palmoplantar pustulosis. From these results, SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome with associated hypertrophic pachymeningitis was diagnosed. After corticosteroid therapy and tonsillectomy, the clinical symptoms and radiological abnormalities were improved. Clinicians should be aware of SAPHO as a potential unusual cause of hypertrophic pachymeningitis.

Introduction

SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome was proposed by Charmot et al. in 1987 [1], to describe a clinicoradiological entity manifesting in the joints, skin, and bones. The fundamental component of SAPHO syndrome is osteitis, usually with negative bacterial cultures, with palmoplantar pustulosis and acne as associated skin lesions. Hypertrophic pachymeningitis (HP) is characterized by diffuse or localized thickening of the dura mater with an inflammatory response. It often presents neurological symptoms such as headache, seizure, and focal neurological deficits. Several etiologies, such as rheumatoid arthritis, syphilis, Wegener's granulomatosis, multifocal fibrosclerosis, tuberculosis, and cancer have been documented [2]. However, the association between SAPHO syndrome and HP has rarely been observed. Here, we report a case of SAPHO syndrome complicated by HP.

Section snippets

Case report

In April 2010, a 40-year-old Japanese woman noted non-throbbing headaches accompanied by fever and swelling of the forehead. She had a medical history of eosinophilia during her third decade. In January 2013, she was admitted to our hospital for a detailed workup. On admission, she reported a severe headache and swelling on her forehead (Fig. 1A and B). Red pustules on her bilateral palms and soles, with mild tenderness (Fig. 1C and D), were noted. No swelling of the tear, parotid or

Discussion

The first diagnostic criteria of SAPHO syndrome were proposed by Benhamou et al. in 1988 [4], updated by Kahn et al. with exclusion criteria in 2003 [5], and are currently widely accepted in clinical practice. In our patient, Kahn's criteria are fulfilled and none of the exclusion criteria apply. While HP is commonly associated with granulomatous disease [2], none of such disorders could be found in our patient. Her serological profile showed a mild elevation of serum IgG4, but IgG4 related

Conflict of interest

All authors declare that this case report involves no conflicts of interest.

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1

Tel.: + 81 93 641 5111; fax: + 81 93 642 1868.

2

Tel.: + 81 92 642 5340; fax: + 81 92 642 5352.

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