- Email: [email protected]
Misleading clavicular osteolytic lesion revealing fibrous dysplasia
Letters to the Editor / Joint Bone Spine 73 (2006) 474–483
With appropriate treatment, recovery is usually satisfactory, most notably at the extensor tendons of the hands [14]. However, recurrences are common and prolonged follow-up should therefore be provided [4,5]. Rehabilitation therapy improves the functional outcome [1].
475
Received 29 December 2004; accepted 3 August 2005 Available online 04 January 2006 *Corresponding
author. Tel.: +212 21 237 781714; fax: +212 21 237 883327.
4. Conclusion We report a highly unusual case of multifocal tuberculous tenosynovitis. Clinical and radiological findings may suggest the diagnosis, which should be confirmed by bacteriological studies and, above all, histological studies. A combination of medical and surgical treatments ensures satisfactory functional outcomes. However, the high risk of recurrences requires prolonged follow-up.
1297-319X/$ - see front matter © Elsevier SAS. All rights reserved. doi:10.1016/j.jbspin.2005.08.002
Misleading clavicular osteolytic lesion revealing fibrous dysplasia Keywords: Fibrous dysplasia; Osteolysis; Bone biopsy; Bisphosphonates
References
1. Introduction
[1]
Fibrous dysplasia is a sporadic congenital disease in which fibrous tissue containing bone trabeculae proliferates within the medullary space [1]. The resulting alteration in bone texture can cause pain, deformities, fractures, and neurological impairment [2,3]. Radiological findings vary widely but are usually highly suggestive. Diagnostic bone biopsy is usually unnecessary, except in monostotic forms with inconclusive imaging study findings and in patients who have pain or radiological changes consistent with malignant transformation [4]. We report a case of fibrous dysplasia with atypical radiological findings.
[2] [3] [4] [5] [6]
[7]
[8] [9]
[10]
[11] [12] [13] [14]
Gester. Affections des gaines synoviales. Encycl Med Chir Appareil locomoteur. 1987. 15153 A10, 9: 8p. Walker UA, Gutfleisch J, Peter H. Case number 23: Tuberculous tenosynovitis. Ann Rheum Dis 2002;61:384. Jackson RH, King JW. Tenosynovitis of the hand: a forgotten manifestation of tuberculosis. Rev Infect Dis 1989;11:616–8. Ortiz E, Moro MJ, Diaz-Curiel M. Chronic otitis and tenosynovitis in an elderly diabetic woman. Postgrad Med 1999;75:121–3. Regnard PJ, Barry P, Isselin J. Mycobacterial tenosynovitis of the flexor tendons of the hand. A report of five cases. J Hand Surg 1996;21:351–4. Hooker MS, Schafer RA, Fishbain JT, Belnap CM. Tuberculous tenosynovitis of the tibial anterior tendon. A case report. Foot Ankle Int 2002; 23:1131–4. Fnini S, Ouarab M, Rafai M, Cohen D, Largab A, Trafeh M. An uncommon occupational accident: tuberculous tenosynovitis of the extensor of the hand. Chir Main 1999;18:309–12. Aubert JP, Stein A, Raoult D, Magalon G. Flexor tenosynovitis in the hand. An unusual aetiology. J Hand Surg [Br] 1995;20:509–10. Morvan G, Busson J, Wybier M, Mathieu PH. In: Pathologie du pied et de la cheville. Imagerie ostéo-articulaire. Paris: Editions Flammarion; 1999. p. 1183–276. Sueyoshi E, Uetani M, Hayashi K, Kohzaki S. Tuberculous tenosynovitis of the wrist: MRI findings in three patients. Skeletal Radiol 1996;32: 279–85. Mateo L, Ruffi G, Nolla JM, Alcaide F. Mycobacterium chelonae tenosynovitis of the hand. Semin Arthritis Rheum 2004;34:617–22. Aboudola S, Sienko A, Carey RB, Johnson S. Tuberculosis tenosynovitis. Hum Path 2004;35:1044–6. Cramer K, Seiler JG, Milek MA. Tuberculous tenosynovitis of the wrist. Two case reports. Clin Orthop Relat Res 1991:137–40. Tsuduki E, Kawada H, Takeda Y, Toyoda E, Kobayashi N, Kudo K, et al. A case of multiple bone and joint tuberculosis which had been misdiagnosed as the rheumatoid arthritis and treated with prednisolone for eleven months. Kekkaku 2002;77:361–6.
Bouchra Amine* Karima Benbouazza Rachid Bahiri Najia Hajjaj-Hassouni Rheumatology B Department (Professor N. Hajjaj-Hassouni), El Ayachi Hospital, Ibn Sina Teaching Hospitals, Rabat-Salé, Morocco E-mail address: [email protected] (B. Amine).
2. Case report A 40-year-old woman with an unremarkable medical history was admitted to our Rheumatology Department in September 2003 for evaluation of a spontaneous fracture of the left clavicle. She reported the abrupt onset in February 2003 of mechanical neck pain with intermittent and poorly systematized radiation to the left arm. The pain was somewhat alleviated by nonsteroidal anti-inflammatory drugs and standard analgesics but worsened in late September 2003 after a sudden movement. Radiographs obtained at pain onset showed a fracture of the left clavicle and degenerative disease at C5-C6. At admission, she was in good general health and had normal range of motion of the cervical spine. The left clavicle was tender to pressure but the overlying tissues showed no evidence of inflammation. Findings from the rest of the physical examination were unremarkable. Plain radiographs centered on the upper thorax disclosed a moth-eaten appearance of the left clavicle and first left rib, with hypertrophy and breaks in the cortex (Fig. 1). A bone scan showed increased uptake at both sites; no other hot spots were visible. The findings did not support Gorham disease or massive idiopathic osteolysis, particularly as flat bones in the thorax were involved. Findings were normal from computed tomography (CT) of the chest, abdomen, and pelvis, as well as from ultrasonography of the neck. CT of the left clavicle and first rib visualized a heterogeneous appearance with osteolytic foci and cortical violation (Fig. 2). Magnetic resonance imaging (MRI) centered on these two sites disclosed low signal on T1 images and high signal on
476
Letters to the Editor / Joint Bone Spine 73 (2006) 474–483
Fig. 1. Plain radiograph: moth-eaten appearance of the bone tissue and cortical breaks in the left clavicle and left first rib. Fig. 3. Plain radiograph dramatic improvement in the appearance of the left clavicle and first rib after pamidronate therapy from October 2003 to June 2004. Note the thickening of the cortices and filling of the osteolytic lesions.
Fig. 2. CT: heterogeneous appearance of the clavicle with osteolytic foci and cortical violation.
T2 images, without soft tissue involvement. The erythrocyte sedimentation rate was 10 mm/h and findings were normal from blood counts and thyroid function tests. Tumor marker assays were negative. Calcium and phosphate levels were normal. Elevations were noted in serum alkaline phosphatase (142 IU/l; normal, 35–120 IU/l) and carboxy-terminal peptide of collagen I (687 pg/ml), whereas osteocalcin was normal (20 ng/ml). The inconclusive nature of the clinical, laboratory, and imaging study findings prompted a bone microbiopsy, which showed nonspecific granulation tissue without evidence of malignancy. A surgical bone biopsy ruled out a malignancy and established the diagnosis of fibrous dysplasia with a callus and formation of fibrous and bony tissue to repair a fracture. Intravenous pamidronate was given in a dosage of 60 mg/day intravenously for 3 consecutive days every 6 months, in combination with vitamin D and calcium supplements. Marked improvements in the clinical symptoms, laboratory test abnormalities, and imaging study findings were noted (Fig. 3). 3. Discussion Fibrous dysplasia has been estimated to contribute 1% of all primary bone tumors and 7% of benign bone tumors [2]. The disease is monostotic in 70% of cases and polyostotic in 30%. Endocrine abnormalities and café-au-lait spots are present in 3% of patients (McCune–Albright syndrome) [5]. Fibrous dys-
plasia may remain asymptomatic for many years, most notably when a single bone is involved, and the lesions may be found unexpectedly on radiographs obtained for another reason [6,7]. Radiographic changes vary widely. Depending on the extent of fibrous tissue ossification, the lesion may be lucent (fibrous tissue), sclerotic (newly formed bone), or mixed (juxtaposition of fibrous tissue, bone, cysts, and in some cases cartilage). “Cloud of smoke” and ground-glass lesions are suggestive. The contours are usually well-defined and sclerotic. Unusual features in our patient included the heterogeneous moth-eaten appearance of the involved bone, the ill-defined margins of the osteolytic lucencies, and the cortical breaks suggesting a malignancy. The cortex around fibrous dysplasia lesions is usually intact or displaced outward and thinned. Even when the cortex is broken, the absence of periosteal reaction and soft tissue involvement suggest a benign lesion. A few cases similar to ours in which the radiological changes suggested aggressive malignant disease have been reported [8]. CT is rarely useful for the diagnosis of fibrous dysplasia but helps to further evaluate the radiological changes and to predict the fracture risk based on cortical thickness measurements. MRI [9] lacks specificity and is difficult to interpret but can be useful in difficult cases to rule out differentials and malignant transformation [10,11]. In particular, soft tissue involvement, when present, can be analyzed in detail. Interpretation of the bone signals is extremely difficult given the histological heterogeneity of the lesions. In patients with polyostotic disease or McCune–Albright syndrome, the clinical manifestations and radiographic findings are usually sufficiently typical to establish the diagnosis. In monostotic disease, however, a bone biopsy is often needed to rule out other conditions. The histological features may be difficult to interpret when the biopsy fragment is small, which may be the case with radioguided biopsies, or when a callus modifies the local bone structure. When the imaging study findings fail to establish the diagnosis with near certainty, a biopsy must be obtained to rule out a malignancy. In our patient, although two bones were involved, a surgical biopsy was needed because the imaging study findings suggested a malignant tumor. The dramatic clinical and radiographic improvements with thickening of the cortices and filling of the osteolytic lesions under bisphosphonate therapy further support the diagnosis of fibrous dysplasia.
Letters to the Editor / Joint Bone Spine 73 (2006) 474–483
477
References
1. Introduction
[1]
Primary hypertrophic osteoarthropathy (HOA), also know as pachydermoperiostosis or Touraine-Solente-Golé syndrome, accounts for only 3% to 5% of all cases of hypertrophic osteoarthropathy [1,2]. This genetic disease is transmitted on an autosomal dominant basis with variable penetrance and predominance in males [2,3]. Pachydermia, seborrhea, and digital clubbing are the main presenting symptoms [1,4]. Although multifocal periostosis is the most typical osteoarticular manifestation, polyarthralgia, often with a mechanical time pattern, and nondestructive oligoarthritis predominantly located at both knees or ankles may occur [4–7]. Hip disease responsible for joint space narrowing has not been reported. We describe a typical case of primary HOA in which the presence of bilateral hip disease might indicate hip involvement by primary HOA or a concomitant chronic inflammatory joint disease.
Lichtenstein L, Jaffe HL. Fibrous dysplasia of bone. A condition affecting one, several or many bones, the graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism or still other extra skeletal abnormalities. Arch Pathol 1938;33:777–816. [2] Avimadje MA, Goupille P, Zerkak D, Begnard G, Besse JB, Valat J. Atteinte rachidienne isolée au cours d’une dysplasie fibreuse. Rev Rhum 2000;67:77–82. [3] Chapurlat RD. Dysplasie fibreuse des os : aspects cliniques chez l’adulte. Rev Rhum 2003;70:678–80. [4] Laredo JD, Champsaur P, Hamze B. Dysplasie fibreuse des os et dysplasie ostéofibreuse. Ann Radiol (Paris) 1995;38:225–36. [5] Marie P. Dysplasie fibreuse : aspects tissulaires, cellulaires et moléculaires. Rev Rhum 2003;70:681–6. [6] Henry A. Monostotic fibrous dysplasia. J Bone Joint Surg 1969;51B: 300–6. [7] Chapurlat RD, Meunier PJ. Fibrous dysplasia of bone. Baillieres Clin Rheumatol 2000;14:385–98. [8] Yao L, Eckardt JJ, Seeger LL. Fibrous dysplasia associated with cortical bony destruction: CT and MR findings. J Comput Assist Tomogr 1994; 18:91–4. [9] Jee WH, Choi KH, Choe BY, Park JM, Shin KS. Fibrous dysplasia: MR imaging characteristics with radiologic correlation. AJR 1996;167:1523– 7. [10] Schwartz DT, Alpert M. The malignant transformation of fibrous dysplasia. Am J Med Sci 1964;247:1–20. [11] Ruggieri P, Sim FH, Bond JR, Unni KK. Malignancies in fibrous dysplasia. Cancer 1994;73:1411–24.
Houda Mâaroufi* Rheumatology Department B, El Ayachi Hospital, Rabat-Salé Teaching Hospital, 11000 Rabat-Salé, Morocco E-mail address: [email protected] (H. Mâaroufi). Roland D. Chapurlat Rheumatology and Bone Diseases Department, F Pavillion, Édouard-Herriot Teaching Hospital, 69437 Lyon cedex 3, France Boubker Benyahia Rheumatology Department B, El Ayachi Hospital, Rabat-Salé Teaching Hospital, 11000 Rabat-Salé, Morocco Pierre D. Delmas Rheumatology and Bone Diseases Department, F Pavillion, Édouard-Herriot Teaching Hospital, 69437 Lyon cedex 3, France Received 30 March 2005; accepted 4 November 2005 Available online 20 March 2006 *Corresponding
author.
1297-319X/$ - see front matter © Elsevier SAS. All rights reserved. doi:10.1016/j.jbspin.2005.11.012
Primary hypertrophic osteoarthropathy with bilateral destructive hip arthritis Keywords: Pachydermoperiostosis; Rheumatic manifestations; Hip disease
2. Case report A 23-year-old man presented with a 6-year history of mechanical joint pain. He reported broadening of the hands, wrists, feet, ankles, and knees over the last 2 years. Analgesics, nonsteroidal antiinflammatory drugs, and aspiration of the knees followed by glucocorticoid injection had failed to improve the symptoms. Features at admission included facial dysmorphia with cutis verticis gyrata of the forehead, digital clubbing, hypertrophy of the extremities with edema and thickened skin, large effusions in both knees, and moderate motion range limitation of both hips with no pain. The heart and lungs were normal to physical examination. Laboratory tests showed 10,300 leukocytes/mm3 and 11.6 g/dl hemoglobin; the erythrocyte sedimentation rate was 32 mm/h and the C-reactive protein level was 11.2 mg/L. Renal and hepatic function tests were normal, as were calcium and phosphate assays. Fluid aspirated from a knee exhibited mechanical properties, with 310 leukocytes/mm3. Radiographs disclosed diffuse periostosis involving the forearm bones, femurs, legs bones, and tubular bones of the hands and feet. Technetium99 bone scanning showed increased uptake by long bones. A synovial biopsy from a knee exhibited features of mechanical synovitis. A diagnosis of HOA was given. Findings were normal or negative from investigations for a cause, including a chest radiograph, echocardiography, an ultrasound scan of the abdomen and pelvis, and thyroid function tests. In addition, the young age and facial dysmorphia supported a diagnosis of primary HOA. No other cases were identified among family members. A radiograph of the pelvis done to evaluate the hip motion limitation showed joint space narrowing at both hips and normal sacroiliac joints (Fig. 1). Investigations were performed to look for inflammatory joint disease, including a radiograph of the thoracolumbar spine, computed tomography of the sacroiliac joints, rheumatoid factor assays, determination of the HLA B27 type, and an ophthalmologic evaluation. Because of a long waiting time for magnetic resonance imaging, computed tomography of the hips was performed. The results showed an acet-
With appropriate treatment, recovery is usually satisfactory, most notably at the extensor tendons of the hands [14]. However, recurrences are common and prolonged follow-up should therefore be provided [4,5]. Rehabilitation therapy improves the functional outcome [1].
475
Received 29 December 2004; accepted 3 August 2005 Available online 04 January 2006 *Corresponding
author. Tel.: +212 21 237 781714; fax: +212 21 237 883327.
4. Conclusion We report a highly unusual case of multifocal tuberculous tenosynovitis. Clinical and radiological findings may suggest the diagnosis, which should be confirmed by bacteriological studies and, above all, histological studies. A combination of medical and surgical treatments ensures satisfactory functional outcomes. However, the high risk of recurrences requires prolonged follow-up.
1297-319X/$ - see front matter © Elsevier SAS. All rights reserved. doi:10.1016/j.jbspin.2005.08.002
Misleading clavicular osteolytic lesion revealing fibrous dysplasia Keywords: Fibrous dysplasia; Osteolysis; Bone biopsy; Bisphosphonates
References
1. Introduction
[1]
Fibrous dysplasia is a sporadic congenital disease in which fibrous tissue containing bone trabeculae proliferates within the medullary space [1]. The resulting alteration in bone texture can cause pain, deformities, fractures, and neurological impairment [2,3]. Radiological findings vary widely but are usually highly suggestive. Diagnostic bone biopsy is usually unnecessary, except in monostotic forms with inconclusive imaging study findings and in patients who have pain or radiological changes consistent with malignant transformation [4]. We report a case of fibrous dysplasia with atypical radiological findings.
[2] [3] [4] [5] [6]
[7]
[8] [9]
[10]
[11] [12] [13] [14]
Gester. Affections des gaines synoviales. Encycl Med Chir Appareil locomoteur. 1987. 15153 A10, 9: 8p. Walker UA, Gutfleisch J, Peter H. Case number 23: Tuberculous tenosynovitis. Ann Rheum Dis 2002;61:384. Jackson RH, King JW. Tenosynovitis of the hand: a forgotten manifestation of tuberculosis. Rev Infect Dis 1989;11:616–8. Ortiz E, Moro MJ, Diaz-Curiel M. Chronic otitis and tenosynovitis in an elderly diabetic woman. Postgrad Med 1999;75:121–3. Regnard PJ, Barry P, Isselin J. Mycobacterial tenosynovitis of the flexor tendons of the hand. A report of five cases. J Hand Surg 1996;21:351–4. Hooker MS, Schafer RA, Fishbain JT, Belnap CM. Tuberculous tenosynovitis of the tibial anterior tendon. A case report. Foot Ankle Int 2002; 23:1131–4. Fnini S, Ouarab M, Rafai M, Cohen D, Largab A, Trafeh M. An uncommon occupational accident: tuberculous tenosynovitis of the extensor of the hand. Chir Main 1999;18:309–12. Aubert JP, Stein A, Raoult D, Magalon G. Flexor tenosynovitis in the hand. An unusual aetiology. J Hand Surg [Br] 1995;20:509–10. Morvan G, Busson J, Wybier M, Mathieu PH. In: Pathologie du pied et de la cheville. Imagerie ostéo-articulaire. Paris: Editions Flammarion; 1999. p. 1183–276. Sueyoshi E, Uetani M, Hayashi K, Kohzaki S. Tuberculous tenosynovitis of the wrist: MRI findings in three patients. Skeletal Radiol 1996;32: 279–85. Mateo L, Ruffi G, Nolla JM, Alcaide F. Mycobacterium chelonae tenosynovitis of the hand. Semin Arthritis Rheum 2004;34:617–22. Aboudola S, Sienko A, Carey RB, Johnson S. Tuberculosis tenosynovitis. Hum Path 2004;35:1044–6. Cramer K, Seiler JG, Milek MA. Tuberculous tenosynovitis of the wrist. Two case reports. Clin Orthop Relat Res 1991:137–40. Tsuduki E, Kawada H, Takeda Y, Toyoda E, Kobayashi N, Kudo K, et al. A case of multiple bone and joint tuberculosis which had been misdiagnosed as the rheumatoid arthritis and treated with prednisolone for eleven months. Kekkaku 2002;77:361–6.
Bouchra Amine* Karima Benbouazza Rachid Bahiri Najia Hajjaj-Hassouni Rheumatology B Department (Professor N. Hajjaj-Hassouni), El Ayachi Hospital, Ibn Sina Teaching Hospitals, Rabat-Salé, Morocco E-mail address: [email protected] (B. Amine).
2. Case report A 40-year-old woman with an unremarkable medical history was admitted to our Rheumatology Department in September 2003 for evaluation of a spontaneous fracture of the left clavicle. She reported the abrupt onset in February 2003 of mechanical neck pain with intermittent and poorly systematized radiation to the left arm. The pain was somewhat alleviated by nonsteroidal anti-inflammatory drugs and standard analgesics but worsened in late September 2003 after a sudden movement. Radiographs obtained at pain onset showed a fracture of the left clavicle and degenerative disease at C5-C6. At admission, she was in good general health and had normal range of motion of the cervical spine. The left clavicle was tender to pressure but the overlying tissues showed no evidence of inflammation. Findings from the rest of the physical examination were unremarkable. Plain radiographs centered on the upper thorax disclosed a moth-eaten appearance of the left clavicle and first left rib, with hypertrophy and breaks in the cortex (Fig. 1). A bone scan showed increased uptake at both sites; no other hot spots were visible. The findings did not support Gorham disease or massive idiopathic osteolysis, particularly as flat bones in the thorax were involved. Findings were normal from computed tomography (CT) of the chest, abdomen, and pelvis, as well as from ultrasonography of the neck. CT of the left clavicle and first rib visualized a heterogeneous appearance with osteolytic foci and cortical violation (Fig. 2). Magnetic resonance imaging (MRI) centered on these two sites disclosed low signal on T1 images and high signal on
476
Letters to the Editor / Joint Bone Spine 73 (2006) 474–483
Fig. 1. Plain radiograph: moth-eaten appearance of the bone tissue and cortical breaks in the left clavicle and left first rib. Fig. 3. Plain radiograph dramatic improvement in the appearance of the left clavicle and first rib after pamidronate therapy from October 2003 to June 2004. Note the thickening of the cortices and filling of the osteolytic lesions.
Fig. 2. CT: heterogeneous appearance of the clavicle with osteolytic foci and cortical violation.
T2 images, without soft tissue involvement. The erythrocyte sedimentation rate was 10 mm/h and findings were normal from blood counts and thyroid function tests. Tumor marker assays were negative. Calcium and phosphate levels were normal. Elevations were noted in serum alkaline phosphatase (142 IU/l; normal, 35–120 IU/l) and carboxy-terminal peptide of collagen I (687 pg/ml), whereas osteocalcin was normal (20 ng/ml). The inconclusive nature of the clinical, laboratory, and imaging study findings prompted a bone microbiopsy, which showed nonspecific granulation tissue without evidence of malignancy. A surgical bone biopsy ruled out a malignancy and established the diagnosis of fibrous dysplasia with a callus and formation of fibrous and bony tissue to repair a fracture. Intravenous pamidronate was given in a dosage of 60 mg/day intravenously for 3 consecutive days every 6 months, in combination with vitamin D and calcium supplements. Marked improvements in the clinical symptoms, laboratory test abnormalities, and imaging study findings were noted (Fig. 3). 3. Discussion Fibrous dysplasia has been estimated to contribute 1% of all primary bone tumors and 7% of benign bone tumors [2]. The disease is monostotic in 70% of cases and polyostotic in 30%. Endocrine abnormalities and café-au-lait spots are present in 3% of patients (McCune–Albright syndrome) [5]. Fibrous dys-
plasia may remain asymptomatic for many years, most notably when a single bone is involved, and the lesions may be found unexpectedly on radiographs obtained for another reason [6,7]. Radiographic changes vary widely. Depending on the extent of fibrous tissue ossification, the lesion may be lucent (fibrous tissue), sclerotic (newly formed bone), or mixed (juxtaposition of fibrous tissue, bone, cysts, and in some cases cartilage). “Cloud of smoke” and ground-glass lesions are suggestive. The contours are usually well-defined and sclerotic. Unusual features in our patient included the heterogeneous moth-eaten appearance of the involved bone, the ill-defined margins of the osteolytic lucencies, and the cortical breaks suggesting a malignancy. The cortex around fibrous dysplasia lesions is usually intact or displaced outward and thinned. Even when the cortex is broken, the absence of periosteal reaction and soft tissue involvement suggest a benign lesion. A few cases similar to ours in which the radiological changes suggested aggressive malignant disease have been reported [8]. CT is rarely useful for the diagnosis of fibrous dysplasia but helps to further evaluate the radiological changes and to predict the fracture risk based on cortical thickness measurements. MRI [9] lacks specificity and is difficult to interpret but can be useful in difficult cases to rule out differentials and malignant transformation [10,11]. In particular, soft tissue involvement, when present, can be analyzed in detail. Interpretation of the bone signals is extremely difficult given the histological heterogeneity of the lesions. In patients with polyostotic disease or McCune–Albright syndrome, the clinical manifestations and radiographic findings are usually sufficiently typical to establish the diagnosis. In monostotic disease, however, a bone biopsy is often needed to rule out other conditions. The histological features may be difficult to interpret when the biopsy fragment is small, which may be the case with radioguided biopsies, or when a callus modifies the local bone structure. When the imaging study findings fail to establish the diagnosis with near certainty, a biopsy must be obtained to rule out a malignancy. In our patient, although two bones were involved, a surgical biopsy was needed because the imaging study findings suggested a malignant tumor. The dramatic clinical and radiographic improvements with thickening of the cortices and filling of the osteolytic lesions under bisphosphonate therapy further support the diagnosis of fibrous dysplasia.
Letters to the Editor / Joint Bone Spine 73 (2006) 474–483
477
References
1. Introduction
[1]
Primary hypertrophic osteoarthropathy (HOA), also know as pachydermoperiostosis or Touraine-Solente-Golé syndrome, accounts for only 3% to 5% of all cases of hypertrophic osteoarthropathy [1,2]. This genetic disease is transmitted on an autosomal dominant basis with variable penetrance and predominance in males [2,3]. Pachydermia, seborrhea, and digital clubbing are the main presenting symptoms [1,4]. Although multifocal periostosis is the most typical osteoarticular manifestation, polyarthralgia, often with a mechanical time pattern, and nondestructive oligoarthritis predominantly located at both knees or ankles may occur [4–7]. Hip disease responsible for joint space narrowing has not been reported. We describe a typical case of primary HOA in which the presence of bilateral hip disease might indicate hip involvement by primary HOA or a concomitant chronic inflammatory joint disease.
Lichtenstein L, Jaffe HL. Fibrous dysplasia of bone. A condition affecting one, several or many bones, the graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism or still other extra skeletal abnormalities. Arch Pathol 1938;33:777–816. [2] Avimadje MA, Goupille P, Zerkak D, Begnard G, Besse JB, Valat J. Atteinte rachidienne isolée au cours d’une dysplasie fibreuse. Rev Rhum 2000;67:77–82. [3] Chapurlat RD. Dysplasie fibreuse des os : aspects cliniques chez l’adulte. Rev Rhum 2003;70:678–80. [4] Laredo JD, Champsaur P, Hamze B. Dysplasie fibreuse des os et dysplasie ostéofibreuse. Ann Radiol (Paris) 1995;38:225–36. [5] Marie P. Dysplasie fibreuse : aspects tissulaires, cellulaires et moléculaires. Rev Rhum 2003;70:681–6. [6] Henry A. Monostotic fibrous dysplasia. J Bone Joint Surg 1969;51B: 300–6. [7] Chapurlat RD, Meunier PJ. Fibrous dysplasia of bone. Baillieres Clin Rheumatol 2000;14:385–98. [8] Yao L, Eckardt JJ, Seeger LL. Fibrous dysplasia associated with cortical bony destruction: CT and MR findings. J Comput Assist Tomogr 1994; 18:91–4. [9] Jee WH, Choi KH, Choe BY, Park JM, Shin KS. Fibrous dysplasia: MR imaging characteristics with radiologic correlation. AJR 1996;167:1523– 7. [10] Schwartz DT, Alpert M. The malignant transformation of fibrous dysplasia. Am J Med Sci 1964;247:1–20. [11] Ruggieri P, Sim FH, Bond JR, Unni KK. Malignancies in fibrous dysplasia. Cancer 1994;73:1411–24.
Houda Mâaroufi* Rheumatology Department B, El Ayachi Hospital, Rabat-Salé Teaching Hospital, 11000 Rabat-Salé, Morocco E-mail address: [email protected] (H. Mâaroufi). Roland D. Chapurlat Rheumatology and Bone Diseases Department, F Pavillion, Édouard-Herriot Teaching Hospital, 69437 Lyon cedex 3, France Boubker Benyahia Rheumatology Department B, El Ayachi Hospital, Rabat-Salé Teaching Hospital, 11000 Rabat-Salé, Morocco Pierre D. Delmas Rheumatology and Bone Diseases Department, F Pavillion, Édouard-Herriot Teaching Hospital, 69437 Lyon cedex 3, France Received 30 March 2005; accepted 4 November 2005 Available online 20 March 2006 *Corresponding
author.
1297-319X/$ - see front matter © Elsevier SAS. All rights reserved. doi:10.1016/j.jbspin.2005.11.012
Primary hypertrophic osteoarthropathy with bilateral destructive hip arthritis Keywords: Pachydermoperiostosis; Rheumatic manifestations; Hip disease
2. Case report A 23-year-old man presented with a 6-year history of mechanical joint pain. He reported broadening of the hands, wrists, feet, ankles, and knees over the last 2 years. Analgesics, nonsteroidal antiinflammatory drugs, and aspiration of the knees followed by glucocorticoid injection had failed to improve the symptoms. Features at admission included facial dysmorphia with cutis verticis gyrata of the forehead, digital clubbing, hypertrophy of the extremities with edema and thickened skin, large effusions in both knees, and moderate motion range limitation of both hips with no pain. The heart and lungs were normal to physical examination. Laboratory tests showed 10,300 leukocytes/mm3 and 11.6 g/dl hemoglobin; the erythrocyte sedimentation rate was 32 mm/h and the C-reactive protein level was 11.2 mg/L. Renal and hepatic function tests were normal, as were calcium and phosphate assays. Fluid aspirated from a knee exhibited mechanical properties, with 310 leukocytes/mm3. Radiographs disclosed diffuse periostosis involving the forearm bones, femurs, legs bones, and tubular bones of the hands and feet. Technetium99 bone scanning showed increased uptake by long bones. A synovial biopsy from a knee exhibited features of mechanical synovitis. A diagnosis of HOA was given. Findings were normal or negative from investigations for a cause, including a chest radiograph, echocardiography, an ultrasound scan of the abdomen and pelvis, and thyroid function tests. In addition, the young age and facial dysmorphia supported a diagnosis of primary HOA. No other cases were identified among family members. A radiograph of the pelvis done to evaluate the hip motion limitation showed joint space narrowing at both hips and normal sacroiliac joints (Fig. 1). Investigations were performed to look for inflammatory joint disease, including a radiograph of the thoracolumbar spine, computed tomography of the sacroiliac joints, rheumatoid factor assays, determination of the HLA B27 type, and an ophthalmologic evaluation. Because of a long waiting time for magnetic resonance imaging, computed tomography of the hips was performed. The results showed an acet-