title: A case of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome presenting with hypertrophic pachymeningitis.
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.
authors.affiliation: Department of Neurology, JCHO Kyushu Hospital, 8-1 kishinoura, Yahatanishi-ku, Kitakyushu City, Fukuoka 806-8501, Japan; Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan.
authors.forename: Wataru
authors.initials: W
authors.lastname: Shiraishi
authors.affiliation: Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan.
authors.forename: Shintaro
authors.initials: S
authors.lastname: Hayashi
authors.affiliation: Department of Neurology, JCHO Kyushu Hospital, 8-1 kishinoura, Yahatanishi-ku, Kitakyushu City, Fukuoka 806-8501, Japan.
authors.forename: Yasutaka
authors.initials: Y
authors.lastname: Iwanaga
authors.affiliation: Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Murai
authors.affiliation: Department of Neurology, JCHO Kyushu Hospital, 8-1 kishinoura, Yahatanishi-ku, Kitakyushu City, Fukuoka 806-8501, Japan.
authors.forename: Akifumi
authors.initials: A
authors.lastname: Yamamoto
authors.affiliation: Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan. Electronic address: kira@neuro.med.kyushu-u.ac.jp.
authors.forename: Jun-ichi
authors.initials: J
authors.lastname: Kira
mesh_terms: D020083:Acquired Hyperostosis Syndrome / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D000328:Adult; D004388:Dura Mater / Q000473:pathology*; D005260:Female; D005334:Fever / Q000209:etiology; D005546:Forehead / Q000473:pathology; D006261:Headache / Q000209:etiology; D006801:Humans; D006984:Hypertrophy / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008581:Meningitis / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology*; D012886:Skull / Q000473:pathology*
keywords: Corticosteroid therapy; Headache; Hypertrophic pachymeningitis; Palmoplantar pustulosis; SAPHO syndrome; Tonsillectomy