title: [Headache as a manifestation of SAPHO syndrome with a lesion extending to the dura mater, parietal bone, and temporal muscle].
abstract: A 50-year-old woman with a history of palmoplantar pustulosis, femur osteomyelitis, and sterno-costo-clavicular hyperostosis presented with a chronic severe left temporal headache that had progressed during the previous year. Her CRP level was elevated. Cranial images showed Gadolinium-enhancement of the left temporal muscle, left parietal bone and dura mater. (99m)Tc-HMDP scintigram showed increased uptake in the left parietal bone, left sterno-costo-clavicular joint, right femoral head and intervertebral joints. Biopsy of the lesion demonstrated 1) proliferation of connective tissue in both perimysium and endomysium of the temporal muscle with mild inflammatory cell infiltration within the interstitium, 2) marked infiltration of granulocytes to the bone marrow of the parietal bone, 3) necrosis and moderate fibrosis in the interstitium with inflammatory cell infiltration in the parietal bone, and 4) moderate fibrosis and slight infiltration of inflammatory cells in the dura mater. The patient was diagnosed with a cranial lesion of synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome. There was a moderate response to treatment with intravenous steroid pulse therapy and subsequent methotrexate. In a case of headache accompanied by inflammatory response, palmoplantar pustulosis and joint lesions such as hyperostosis, the possibility of a rare cranial manifestation of SAPHO syndrome should be considered.
authors.affiliation: Department of Neurology, Tokyo Metropolitan Neurological Hospital.
authors.forename: Miho
authors.initials: M
authors.lastname: Uematsu
authors.forename: Shinsuke
authors.initials: S
authors.lastname: Tobisawa
authors.forename: Masahiro
authors.initials: M
authors.lastname: Nagao
authors.forename: Shiro
authors.initials: S
authors.lastname: Matsubara
authors.forename: Toshio
authors.initials: T
authors.lastname: Mizutani
authors.forename: Makoto
authors.initials: M
authors.lastname: Shibuya
mesh_terms: D020083:Acquired Hyperostosis Syndrome / Q000150:complications / Q000473:pathology*; D004388:Dura Mater / Q000473:pathology*; D005260:Female; D006261:Headache / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D010294:Parietal Bone / Q000473:pathology*; D013703:Temporal Muscle / Q000473:pathology*