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Lytic lesion of the sternum. Rare manifestation of sarcoidosis
Lytic Lesion of the Sternum Rare Manifestation of Sarcoidosis
THOMAS Syracuse,
J. OVEN, New
M.D.
York
MAURICE SONES, M.D.* WILLIAM L. MORRISSEY, Philadelphia,
M.D.+
Pennsylvania
Increasing tenderness of the sternum developed four years after pulmonary sarcoidosis had been diagnosed in a 34-year-old woman. Sternal tomography and bone scanning demonstrated abnormalities in the area. Sternal biopsy confirmed the diagnosis of osseous sarcoid. Involvement of the sternum by sarcoidosis has not been previously reported. When a patient with proved sarcoidosis presents with clinical evidence of a new bony lesion, other abnormalities must be excluded, since neoplastic, infectious, and metabolic diseases may be roentgenographically indistinguishable from osseous sarcoid. This patient demonstrated several unusual features of sarcoidosis. After three years of quiescence, her disease erupted with multisystem involvement, including retinal vasculitis, anterior uveitis, granulomatous pharyngitis, and lymphadenopathy, while her pulmonary status remained stable. In addition, a bony lesion of the sternum developed and eventually also was proved to be caused by sarcoidosis.
CASE REPORT
From the Medical College of Pennsylvania and Hospital, Philadelphia, Pennsylvania, and Upstate Medical Center, Syracuse, New York. Manuscript accepted November 7, 1984. * Deceased. t Current address and address for reprint requests: Frankford Hospital, Knights and Red Lion Roads, Philadelphia, Pennsylvania 19114.
In December 1975, a 30-year-old black woman presented to the Hospital of the Medical College of Pennsylvania complaining of bilateral lower extremity pain and swelling unresponsive to the local application of heat. When chest roentgenography revealed bilateral hilar adenopathy and gastrocnemius muscle biopsy demonstrated granulomatous myositis, the diagnosis of sarcoidosis was established. The patient was treated with steroids for several months and subsequently did well with no additional therapy until October 1978 when she was admitted to another hospital with blurred vision. She was found to have bilateral severe retinal periphlebitis, retinal hemorrhages, branch vein occlusion, and anterior uveitis. The left eye also demonstrated retinal neovascularization. She was treated with laser photocoagulation and varying doses of prednisone, which stabilized her condition until July 1979. She then complained of anterior chest wall pain increasing over one month and made worse by palpation but not hindering activity. Physical examination revealed only hyperplastic mucosa in the posterior oropharynx, submandibular lymphadenopathy, and tenderness over the mid-sternal area. Oropharyngeal biopsy and muscle biopsy in that area showed granulomatous changes consistent with sarcoidosis. The dosage of prednisone was increased to 60 mg daily. After two months, some resolution of the submandibular adenopathy had occurred but the patient continued to complain of chest pain. Sternal tomography was performed and demonstrated a localized destructive lesion of the mid-portion of the body of the sternum (Figure 1). Radioactive technetium-99m-labeled pyrophosphate bone scan-
February
1988
The American
Journal
of Medicine
Volume
80
285
STERNAL
LYTIC
LESION-OVEN
ET AL
reflecting the presence of chronic fibrotic sarcokfosis in all tissue systems. The bones of the hands and feet are most commonly involved in sarcoidosis; however, other bone involvement has been recognized. In 1972, Bouvier et al [2] reported a case of skull vault sarcoidosis and reviewed six other cases of lytic lesions from the literature. Osseous lesions are also known to occur in the nasal bones, long bones, vertebrae, pelvis, and ribs [3-61. The clinical presentation of patients with osseous sarcoid is highly variable, but it is usually found only in patients with persistent disease. Although a large percentage of these patients are completely asymptomatic, in one study nearly one half of them had pain and stiffness of the digits [7]. In only a few, however, was it sufficient to draw attention to the sarcoidosis. Soft tissue swelling has
Figure 1. Tomogram of the sternum demonstrates a lytic lesion involving the mid-portion of the sternum with a break in the bony cortex.
ning illustrated a corresponding area of increased uptake 2). Results of repeated chest roentgenography at that time were unchanged from previous findings. Routine laboratory data were normal. The patient subsequently underwent open sternal biopsy to rule out neoplastic and infectious processes. The biopsy specimen was interpreted as showing granulomatous osteomyelitis consistent with sarcoidosis. Special stains and cultures for tubercule bacilli and fungi showed no growth. The patient’s prednisone therapy was continued and 300 mg of ibuprofen was added four times per day. Six weeks later, the patient’s condition was clinically stable and she was without complaints. Tapering of steroid therapy was accomplished without incident and she remained asymptomatic for more than 12 months of follow-up. (Figure
COMMENTS
Osseous lesions occurring in sarcoidosis are well established but compared with other systemic manifestations of this disease are less frequent. The frequency of bony involvement in sarcokfosis is reported as between 1 percent and 17 percent with the highest incidence occurring in a large international study [I]. In this survey, bone cysts were also noted in 96 (5 percent) of 2,483 patients,
288
February
1988
The
American
Journal
of Medicine
Volume
Figure 2. radionuclide
80
Bone scan reveals a focal area of increased accumulation in the midline of the manubrium.
STERNAL
been noted to occur over areas of cystic bone involvement in a substantial number of patients, and this finding may precede the roentgenographic diagnosis by months or years. In a small percentage of patients, some degree of permanent deformity occurs. When a single lesion involves one of the large bones, the presentation may be that of exquisite tenderness over the area, as exemplified by our patient. The association between bone lesions and chronic skin lesions, particularly lupus pernio, is well recognized [3]. Some authors believe that bone involvement associated with cutaneous lesions suggests a poor prognosis. James et al [7] reported a mortality rate of 2 1 percent in these patients, which was four times the expected mortality from sarcoidosis. The roentgenographic appearance of osseous sarcoidosis usually consists of well-defined, punched-out cystic lesions involving the phalangeal or metacarpal heads. These same osteolytic lesions may occur in any of the larger bones, as was demonstrated in the present case. Another common radiologic presentation of the disease is a coarsely trabeculated pattern usually affecting the phalanges, thus giving the bone a lace-like or reticular ap-
LYTIC
LESION-OVEN
ET AL
pearance. Much less commonly, sarcoidosis can produce osteosclerotic lesions and these have been described in the pelvis, vertebrae, femur, tibia, ribs, skull, and sphenoid bones [8-lo]. In one study, bone surveys were obtained in 10 patients with sarcoidosis who had prior involvement of the bones of the hands or feet [I 01. No further lesions were demonstrated, suggesting that roentgenographic studies are of low diagnostic yield in patients with sarcoidosis but lacking localized bony symptoms. Bone scanning using radioactive technetium-99m-labeled pyrophosphate has only occasionally been reported in osseous sarcoidosis, but is thought to be more sensitive than either radiography or gallium-67 citrate in detecting skeletal involvement [ 1 I]. The roentgenographic abnormalities are not diagnostic in osseous sarcoid. Solid tumor metastases, lymphoma, Hodgkin’s disease, multiple myeloma, pyogenic or tuberculous infections, bone infarction, and Paget’s disease must also be considered. With unusual presentations, as in the present case, open biopsy may be necessary to establish the diagnosis.
REFERENCES 1. 2.
3. 4. 5. 6.
James DG, Neville E, Siltzbach LE, et al: A world wide review of sarcoidosis. Ann NY Acad Sci 1976; 278: 321-334. Bouvier M, Lejeunne E, Quensau P, Ryan M: Sarcoidosis avec lacunes cranieres. Rev Rhum Mal Osteoartic 1972; 39: 205-2 14. Curtis GT: Sarcoidosis of the nasal bones. Br J Radio1 1964; 37: 68-70. Turek SL: Sarcoid disease of bone at the ankle joint. J Bone Joint Surg [Am] 1953; 35: 465-468. Berk RN, grower TD: Vertebral sarcoidosis. Radiology 1964; 82: 660-663. Bloch S, Movson IJ, Seedar YK: Unusual skeletal manifestations in a case of sarcoidosis. Clin Radio1 1968; 19:
February
7. 8. 9.
10.
11.
1986
226. James G, Neville E, Carstairs LS: Bone and joint sarcoidosis. Semin Arthritis Rheum 1976; 6: 53-81. Young DA, Laman ML: Radiodense skeletal lesions in Boeck’s sarcoid. AJR 1972; 114: 553-558. Lin S-R, Levy W, Geo EG, et al: Unusual osteosclerotic changes in sarcoidosis, simulating osteoblastic metastasis. Radiology 1973; 106: 311-312. Stein GN, Israel HL, Sones M: A roentgenographic study of skeletal lesions in sarcoidosis. Arch Intern Med 1956; 97: 532-536. Rohatgi PK: Radioisotope scanning in osseous sarcoidosis. AJR 1980; 134: 189-191.
The American
Journal
of Medicine
Volume
60
267
THOMAS Syracuse,
J. OVEN, New
M.D.
York
MAURICE SONES, M.D.* WILLIAM L. MORRISSEY, Philadelphia,
M.D.+
Pennsylvania
Increasing tenderness of the sternum developed four years after pulmonary sarcoidosis had been diagnosed in a 34-year-old woman. Sternal tomography and bone scanning demonstrated abnormalities in the area. Sternal biopsy confirmed the diagnosis of osseous sarcoid. Involvement of the sternum by sarcoidosis has not been previously reported. When a patient with proved sarcoidosis presents with clinical evidence of a new bony lesion, other abnormalities must be excluded, since neoplastic, infectious, and metabolic diseases may be roentgenographically indistinguishable from osseous sarcoid. This patient demonstrated several unusual features of sarcoidosis. After three years of quiescence, her disease erupted with multisystem involvement, including retinal vasculitis, anterior uveitis, granulomatous pharyngitis, and lymphadenopathy, while her pulmonary status remained stable. In addition, a bony lesion of the sternum developed and eventually also was proved to be caused by sarcoidosis.
CASE REPORT
From the Medical College of Pennsylvania and Hospital, Philadelphia, Pennsylvania, and Upstate Medical Center, Syracuse, New York. Manuscript accepted November 7, 1984. * Deceased. t Current address and address for reprint requests: Frankford Hospital, Knights and Red Lion Roads, Philadelphia, Pennsylvania 19114.
In December 1975, a 30-year-old black woman presented to the Hospital of the Medical College of Pennsylvania complaining of bilateral lower extremity pain and swelling unresponsive to the local application of heat. When chest roentgenography revealed bilateral hilar adenopathy and gastrocnemius muscle biopsy demonstrated granulomatous myositis, the diagnosis of sarcoidosis was established. The patient was treated with steroids for several months and subsequently did well with no additional therapy until October 1978 when she was admitted to another hospital with blurred vision. She was found to have bilateral severe retinal periphlebitis, retinal hemorrhages, branch vein occlusion, and anterior uveitis. The left eye also demonstrated retinal neovascularization. She was treated with laser photocoagulation and varying doses of prednisone, which stabilized her condition until July 1979. She then complained of anterior chest wall pain increasing over one month and made worse by palpation but not hindering activity. Physical examination revealed only hyperplastic mucosa in the posterior oropharynx, submandibular lymphadenopathy, and tenderness over the mid-sternal area. Oropharyngeal biopsy and muscle biopsy in that area showed granulomatous changes consistent with sarcoidosis. The dosage of prednisone was increased to 60 mg daily. After two months, some resolution of the submandibular adenopathy had occurred but the patient continued to complain of chest pain. Sternal tomography was performed and demonstrated a localized destructive lesion of the mid-portion of the body of the sternum (Figure 1). Radioactive technetium-99m-labeled pyrophosphate bone scan-
February
1988
The American
Journal
of Medicine
Volume
80
285
STERNAL
LYTIC
LESION-OVEN
ET AL
reflecting the presence of chronic fibrotic sarcokfosis in all tissue systems. The bones of the hands and feet are most commonly involved in sarcoidosis; however, other bone involvement has been recognized. In 1972, Bouvier et al [2] reported a case of skull vault sarcoidosis and reviewed six other cases of lytic lesions from the literature. Osseous lesions are also known to occur in the nasal bones, long bones, vertebrae, pelvis, and ribs [3-61. The clinical presentation of patients with osseous sarcoid is highly variable, but it is usually found only in patients with persistent disease. Although a large percentage of these patients are completely asymptomatic, in one study nearly one half of them had pain and stiffness of the digits [7]. In only a few, however, was it sufficient to draw attention to the sarcoidosis. Soft tissue swelling has
Figure 1. Tomogram of the sternum demonstrates a lytic lesion involving the mid-portion of the sternum with a break in the bony cortex.
ning illustrated a corresponding area of increased uptake 2). Results of repeated chest roentgenography at that time were unchanged from previous findings. Routine laboratory data were normal. The patient subsequently underwent open sternal biopsy to rule out neoplastic and infectious processes. The biopsy specimen was interpreted as showing granulomatous osteomyelitis consistent with sarcoidosis. Special stains and cultures for tubercule bacilli and fungi showed no growth. The patient’s prednisone therapy was continued and 300 mg of ibuprofen was added four times per day. Six weeks later, the patient’s condition was clinically stable and she was without complaints. Tapering of steroid therapy was accomplished without incident and she remained asymptomatic for more than 12 months of follow-up. (Figure
COMMENTS
Osseous lesions occurring in sarcoidosis are well established but compared with other systemic manifestations of this disease are less frequent. The frequency of bony involvement in sarcokfosis is reported as between 1 percent and 17 percent with the highest incidence occurring in a large international study [I]. In this survey, bone cysts were also noted in 96 (5 percent) of 2,483 patients,
288
February
1988
The
American
Journal
of Medicine
Volume
Figure 2. radionuclide
80
Bone scan reveals a focal area of increased accumulation in the midline of the manubrium.
STERNAL
been noted to occur over areas of cystic bone involvement in a substantial number of patients, and this finding may precede the roentgenographic diagnosis by months or years. In a small percentage of patients, some degree of permanent deformity occurs. When a single lesion involves one of the large bones, the presentation may be that of exquisite tenderness over the area, as exemplified by our patient. The association between bone lesions and chronic skin lesions, particularly lupus pernio, is well recognized [3]. Some authors believe that bone involvement associated with cutaneous lesions suggests a poor prognosis. James et al [7] reported a mortality rate of 2 1 percent in these patients, which was four times the expected mortality from sarcoidosis. The roentgenographic appearance of osseous sarcoidosis usually consists of well-defined, punched-out cystic lesions involving the phalangeal or metacarpal heads. These same osteolytic lesions may occur in any of the larger bones, as was demonstrated in the present case. Another common radiologic presentation of the disease is a coarsely trabeculated pattern usually affecting the phalanges, thus giving the bone a lace-like or reticular ap-
LYTIC
LESION-OVEN
ET AL
pearance. Much less commonly, sarcoidosis can produce osteosclerotic lesions and these have been described in the pelvis, vertebrae, femur, tibia, ribs, skull, and sphenoid bones [8-lo]. In one study, bone surveys were obtained in 10 patients with sarcoidosis who had prior involvement of the bones of the hands or feet [I 01. No further lesions were demonstrated, suggesting that roentgenographic studies are of low diagnostic yield in patients with sarcoidosis but lacking localized bony symptoms. Bone scanning using radioactive technetium-99m-labeled pyrophosphate has only occasionally been reported in osseous sarcoidosis, but is thought to be more sensitive than either radiography or gallium-67 citrate in detecting skeletal involvement [ 1 I]. The roentgenographic abnormalities are not diagnostic in osseous sarcoid. Solid tumor metastases, lymphoma, Hodgkin’s disease, multiple myeloma, pyogenic or tuberculous infections, bone infarction, and Paget’s disease must also be considered. With unusual presentations, as in the present case, open biopsy may be necessary to establish the diagnosis.
REFERENCES 1. 2.
3. 4. 5. 6.
James DG, Neville E, Siltzbach LE, et al: A world wide review of sarcoidosis. Ann NY Acad Sci 1976; 278: 321-334. Bouvier M, Lejeunne E, Quensau P, Ryan M: Sarcoidosis avec lacunes cranieres. Rev Rhum Mal Osteoartic 1972; 39: 205-2 14. Curtis GT: Sarcoidosis of the nasal bones. Br J Radio1 1964; 37: 68-70. Turek SL: Sarcoid disease of bone at the ankle joint. J Bone Joint Surg [Am] 1953; 35: 465-468. Berk RN, grower TD: Vertebral sarcoidosis. Radiology 1964; 82: 660-663. Bloch S, Movson IJ, Seedar YK: Unusual skeletal manifestations in a case of sarcoidosis. Clin Radio1 1968; 19:
February
7. 8. 9.
10.
11.
1986
226. James G, Neville E, Carstairs LS: Bone and joint sarcoidosis. Semin Arthritis Rheum 1976; 6: 53-81. Young DA, Laman ML: Radiodense skeletal lesions in Boeck’s sarcoid. AJR 1972; 114: 553-558. Lin S-R, Levy W, Geo EG, et al: Unusual osteosclerotic changes in sarcoidosis, simulating osteoblastic metastasis. Radiology 1973; 106: 311-312. Stein GN, Israel HL, Sones M: A roentgenographic study of skeletal lesions in sarcoidosis. Arch Intern Med 1956; 97: 532-536. Rohatgi PK: Radioisotope scanning in osseous sarcoidosis. AJR 1980; 134: 189-191.
The American
Journal
of Medicine
Volume
60
267