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CASE REPORT
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Recurrent Unilateral Headache Associated with SAPHO Syndrome Jun Tsugawa, Shinji Ouma, Jiro Fukae and Yoshio Tsuboi
Abstract A 57-year-old woman was admitted with recurrent episodes of right frontal headache. Head magnetic resonance imaging (MRI) revealed extensive thickening and enhancement of the right frontal dura, muscle and fascia, as well as abnormal signal intensity and enhancement of bone marrow at the lesions. Synovitis-acnepustulosis-hyperostosis osteomyelitis (SAPHO) syndrome was diagnosed based on the patient’s 8-year history of treatment of palmoplantar pustulosis and abnormal accumulations in the right temporal, sternum, and left medial clavicula on bone scintigraphy. SAPHO syndrome may be associated with skull lesions, which can contribute to the onset of repeated headache or dural thickening, thus these symptoms should be recognized as manifestations of this syndrome. Key words: SAPHO syndrome, headache, temporal osteomyelitis, dural thickening (Intern Med 53: 1559-1562, 2014) (DOI: 10.2169/internalmedicine.53.2150)
Introduction Palmoplantar pustulosis presents as multiple sterile pustules on the palms and soles. It is sometimes associated with chronic recurrent bone lesions, such as arthritis, especially involving the articulatio sternoclavicularis and spondylitis, and such conditions are called synovitis-acne-pustulosishyperostosis osteomyelitis (SAPHO) syndrome. The main characteristic feature is lesions most frequently located on the anterior chest wall; however, the syndrome is occasionally associated with skull lesions that may result in recurrent headache episodes of unknown origin. There have also been reported cases of SAPHO syndrome with intracranial lesions, including cranial nerve palsy and thickening of the dura mater (1, 2). We herein describe a case of SAPHO syndrome with repeated unilateral headaches and dura mater thickening.
Case Report A 57-year-old woman complained with recurrent episodes of right frontal headache. She had suffered from palmoplantar pustulosis since the age of 49. The patient noted the on-
set of right frontal headache, which was not throbbing, in April of 2009, and swelling presented in the subcutaneous tissue of the painful area on the next day. She was prescribed an analgesic antipyretic, which resolved the symptoms in a few days. In August, swelling and pain in the right frontal region reappeared and then disappeared spontaneously within 2 or 3 days. She had similar symptoms again in around November, but they spontaneously improved. In December, she experienced the most severe pain from the right fronto-temporal region to the ear. Magnetic resonance imaging (MRI) of the head, performed at a local hospital, showed muscle swelling and dural thickening of the right temporalis. In January of 2010, she was referred to our hospital. On admission, the patient had normal vital signs. Papules, pustules, and scales were visible on the palms and soles (Fig. 1). The right fronto-temporal region was slightly bulging and tender without superficial temporal artery engorgement. Neurological examination revealed that her consciousness was clear, and the cranial nerves were intact. Papilledema and meningeal irritation were not found, and we observed no motor or sensory abnormalities or incoordination deficits. The deep tendon reflexes of the extremities were normoactive.
Department of Neurology, Faculty of Medicine, Fukuoka University, Japan Received for publication November 18, 2013; Accepted for publication January 26, 2014 Correspondence to Dr. Yoshio Tsuboi,
[email protected]
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DOI: 10.2169/internalmedicine.53.2150
Laboratory findings on admission: The complete blood count findings were within normal limits. Biochemical tests showed hypoalbuminemia and mild inflammation with total protein of 6.5 g/dL, albumin of 3.4 g/dL, and C-reactive protein of 0.5 mg/dL. Serum IgG4 level was not evaluated. The coagulation tests were normal. There was a slight increase in erythrocyte sedimentation rate [28 mm (60 min) and 49 mm (120 min)]. Autoimmunity tests for antinuclear antibody, rheumatoid factor, anti SS-A antibody, anti SS-B antibody, proteinase 3 specific antineutrophil cytoplasmic antibody (PR3-ANCA) and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) were all negative. Cerebrospinal fluid (CSF) examination was normal with CSF opening pressure of 120 mmH2O and an IgG index of 0.46. Bacterial and fungal cultures of the CSF were negative, and cytological study of the CSF was normal.
Figure 1. Palmoplantar pustulosis. Aseptic pustular and hyperkeratotic lesions affecting the patient’s palmar surface were suggestive of palmoplantar pustulosis.
MRI and scintigraphy findings: Head MRI with T1weighted images (T1WI) (Fig. 2A, B) revealed thickening of the right fronto-temporal muscle/fascia, and post-contrast T1WI with Gd showed marked enhancement in the thickened regions. The bone marrow of the skull affected by lesions exhibited slightly increased signal on T2WI and slight enhancement on post-contrast T1WI. The contrasted T1WI also exhibited extensive dural thickening and enhancement, especially below the internal lamina of the right frontotemporal bones, which showed relatively significant hyperintensity on T2W1. The bone scintigraphy with 99mTechnetium (99mTc) complexes (Fig. 3) revealed abnormal accumulations in the right os temporal, sternum, and the left medial clavicula. Fludeoxyglucose positron emission tomography (FDG-PET) examination was not performed. Clinical course: Nonsteroidal anti-inflammatory drugs (NSAIDs) were administered to alleviate the symptoms of right temporal pain and swelling. The pain subsided but persisted. Because of dural thickening proximal to the site of pain observed on the head MRI, laboratory testing was conducted to search for the cause of hypertrophic pachymeningitis. Idiopathic hypertrophic pachymeningitis, tuberculous meningitis, sarcoidosis, ANCA-associated vasculitis, and malignant lymphoma were also considered possibilities, but none of these diagnoses could be established based on the normal blood examination results (except for mild inflammatory changes and hypoalbuminemia associated with chronic inflammation), the normal CSF findings and the lack of relevant findings on the work-up of the chest and abdomen. The contrasted head MRI showed abnormal signal intensity and Gd-enhancement in the bones, muscle, fascia, and dural lesions. Skeletal scintigraphy revealed abnormal accumulations in the right os temporal, sternum, and left medial clavicula. In our case, a diagnosis of SAPHO syndrome was most likely on the basis of an 8-year history of
A
B
Figure 2. Brain MRI on admission. A contrast-enhanced axial T1-weighted MR image (A) revealed enhancement of the dura matter in the right front-temporal region. Axial T2-weighted MR images (B) demonstrated myofascial thickening in the right fronto-temporal regions and low signal intensities in bone marrow on the same side. 1560
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Figure 3. Bone scintigraphy with 99mTc complexes. 99mTc bone scintigraphy showing increased uptake in the sternocostoclavicular regions and the right temporal skull base.
treatment of palmoplantar pustulosis, her 1-year history of unspecified sternal osteomyelitis, and the skeletal scintigraphy findings. Prednisolone 15 mg daily was initiated for treatment of the disease, and improvement of headache and palmoplantar pustulosis followed. She has remained free of similar symptoms for 6 months.
Discussion The patient was diagnosed with recurrent osteoarthritis (SAPHO syndrome) in association with palmoplantar pustulosis, based on characteristic imaging findings, the past history of sterna osteomyelitis, and her medical history including repeated headache episodes. SAPHO syndrome was first described in 1987. The acronym “SAPHO” describes the combination of synovitis, acne, pustulosis, hyperostosis, and osteitis, and was coined to represent the pathologic conditions that have previously been reported under a variety of names, such as sternocostoclavicular hyperostosis and pustulotic arthro-osteitis (3). The pathogenic mechanism of SAPHO syndrome is not completely understood. It has been suggested that the disease resulted from HLA-B27-related immunopathy because antigen HLA B-27 was more prevalent in patients with skin involvement (4). The present case thus fulfills Kahn’s three diagnostic criteria (5). SAPHO syndrome is commonly thought to be characterized by lesions involving the anterior chest, including the costoclavicular and sternoclavicular joints. The typical scintigraphic manifestation of the syndrome includes tracer uptake of the articulation sternoclavicularis with the manubrium sterni, called the “bullhead sign,” with high sen-
Figure 4. Fat-suppression MRI of the sternum performed 1 year before hospitalization. One year before admission when she diagnosed sternal osteomyelitis, fat-suppression MR imaging (short TI inversion recovery, STIR) showed high signal intensities in the sternum, suggesting the presence of edematous/ inflammatory changes.
sitivity and specificity for the diagnosis of SAPHO syndrome (Fig. 3) (6). Fat-suppression MRI (Fig. 4) performed 1 year before hospitalization revealed high signal intensity from the manubrium to the corps sterni; in addition, the skeletal scintigraphy performed at the hospital showed abnormal accumulations in the same sites. These results indicate that our case was likely to have anterior chest lesions characteristic of SAPHO syndrome. The present patient had an unusual clinical course of SAPHO syndrome in which temporal lesions contributed to repeated headache episodes. Besides the anterior chest lesions, SAPHO syndrome is known to be frequently related to lesions involving the spine (7), articulatio sacroiliaca, os longum, and os plana, as well as peripheral arthritis (8). In addition, a limited number (four cases) of skull bone involvement have been reported (1, 2, 9, 10). In all of these cases except for one with SAPHO syndrome leading to deafness (9), the patient suffered from headache. Thus, in evaluating headache, it is important to differentiate the syndrome from other possible causes of headache. In most cases with headache, the imaging tests showed dural thickening or focal enforcement just below the cranial bone lesions, resembling the images observed in our case. In the case reported by Di Meco et al. (10), a therapeutic incision of the dura was made, and a biopsy of the pathological lesion specimen revealed signs of chronic inflammation. Similar changes were found within the abnormal bone of the skull tissue. An imaging test performed after steroid treatment revealed that enhancement previously seen in the dura had resolved. In SAPHO syndrome with skull involvement,
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as with the abovementioned case, dural thickening and thickened cranial muscle or fascia are considered attributable to a spreading of sterile inflammatory reaction mainly involving bone lesions. The relationship between palmoplantar pustulosis and osteoarticular lesions is not been completely understood. As shown in our case, SAPHO syndrome may be associated with skull lesions; thus, patients with the syndrome should be carefully examined with particular attention given to potential lesions of all sites other than sternocostoclavicular regions. In evaluating headache or dural thickening, SAPHO syndrome should be considered as a possibility, and the presence/absence of palmoplantar pustulosis should be assessed. The authors state that they have no Conflict of Interest (COI).
References 1. Hiwatani Y, Miwa H, Kondo T. Aseptic meningitis as rare manifestation of SAPHO syndrome. Neurology 71: 1645-1647, 2008. 2. Seno A, Arata J, Matsuura Y, Suemitsu I. Palmoplantar pustulosis with pustuloticarthroosteitis involving temporal osteomyelitis and
meningitis. J Dermatol 27: 269-272, 2000. 3. Chamot AM, Benhamou CL, Kahn MF, Beraneck L, Kaplan G, Prost A. Acne-pustulosis-hyperostosis-osteitis syndrome. Result of a national survey. 85 cases. Revue Rhum Maladies Osteoarticulaires 54: 187-196, 1987. 4. Beretta-Piccoli BC, Sauvain MJ, Gal I, et al. Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome in childhood: a report of ten cases and review of the literauture. Eur J Pediatr 159: 594-601, 2000. 5. Kahn MF, Khan MA. The SAPHO syndrome. Baillieres Clin Rheumatol 8: 333-362, 1994. 6. Freyschmidt J, Sternberg A. The bullhead sign: scintigraphic pattern of sternocostoclavicular hyperostosis and pustulotic arthroosteitis. Eur Radiol 8: 807-812, 1998. 7. Laredo JD, Vuillemin-Bodaghi V, Bountry N, Cotten A, ParlierCuau C. SAPHO syndrome: MR appearance of vertebral involvement. Radiology 242: 825-831, 2007. 8. Hayem G, Bouchaud-Chabot A, Benail K, et al. SAPHO syndrome: a long-term follow up study of 120 cases. Semin Arthritis Rheum 29: 159-171, 1999. 9. Marsot-Dupuch K, Doyen JE, Grauer WO, de Givry SC. SAPHO syndrome of the temporomandibular joint associated with sudden deafness. AJNR Am J Neuroradiol 20: 902-905, 1999. 10. DiMeco F, Clatterbuck RE, Li KW, McCarthy EF, Olivi A. Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome presenting as a primary calvarial lesion: case report and review of the literature. J Neurosurg 93: 693-697, 2000.
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