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Osseous sporotrichosis
Journal of Infection (1989) I9, 273-276
CASE REPORTS Osseous sporotrichosis S. G o v e n d e r , M. N. R a s o o l and M. N g c e l w a n e
Department of Orthopaedics, University of Natal Medical School, P.O. Box I7O39, Congella 4o13, Durban, South Africa Accepted for publication 6 May 1989 Summary We report on four patients with osteomyelitis due to Sporothrix schenckii who were successfully treated with potassium iodide. Introduction
Sporothrix schenckii was first isolated in I898 at Johns Hopkins Hospital by Schenck who cultured the fungus from a patient with multiple subcutaneous abscesses. 1 T h e fungus, which is saprophytic, has been found in the soil, shrubs, flowers and in timber. Most often, transmission to h u m a n beings arises through a breach in the skin, especially of the hands and feet during the handling of contaminated vegetation. T h e infection is commonly associated with farmers and gardeners and has been described as 'the syndrome of the alcoholic rose gardener'fl Cutaneous or lymphocutaneous lesions are the commonest manifestations of sporotrichosis. 3 After an incubation period varying from 7 to 40 weeks, the fungus produces a reddish purple nodule. A series of subcutaneous nodules develops along the path of the lymphatic drainage of the primary lesion. A few patients may develop deep-seated abscessesJ In the fixed cutaneous form, the process is restricted to the site of inoculation. T h e histopathological appearance of the nodule is that of a central core of caseous or purulent material surrounded by a zone of polymorphonuclear leucocytes and macrophages. Surrounding this central zone are sheets of epithelioid cells with an intermingling of multinuclear giant cells. Around the periphery, lymphocytes and connective tissue cells are the prominent featuresJ Individual lesions may vary by displaying some or all of the foregoing characteristics. T h e microscopical appearance is not specific for sporotrichosis and indeed cannot be differentiated from tuberculosis and other fungal lesions. Definitive diagnosis rests solely on culture of the organismJ T h e extracutaneous or systemic forms of the disease may follow the cutaneous lesions or arise in their absence. T h e y involve the lungs, musculoskeletal system 5 and other sites. Case reports Case x An 8-year-old boy presented with pain of 8 weeks' duration over the distal part of the left forearm. A lesion over the distal part of the left ulna had been o163-4453/89/o6o273 + o 4 $o2.oo/o
© 1989 The British Society for the Study of Infection 12-2
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S. GOVENDER E T A L .
biopsied at a peripheral hospital. T h e biopsy had been reported as showing evidence of chronic non-specific infection. Clinically, the distal part of the forearm was tender over the ulna border. Apart from a well-healed biopsy scar there was no evidence of any cutaneous lesion or nodal involvement. Radiographs revealed a lytic lesion of the distal part of the ulna with periosteal new bone formation (Plate I). Apart from an ESR of 6o m m / h , all other haematological investigations were normal. Thorough curettage of the lesion yielded material from which S. schenckii grew in culture. T h e child was treated with potassium iodide for 4 weeks with complete resolution of thelesion (Plate 2). Case 2
A 29-year-old man presented with pain over the shin of the left leg of 4 months' duration. A year earlier he had fallen and sustained a minor abrasion over the tibia, which healed well. Clinically, he was tender over the tibia but without skin lesions. T h e ESR was 47 m m / h and a radiograph of the left tibia showed a lytic lesion with a well-defined zone of sclerosis. T h e appearance suggested a Brodie's abscess or an osteoid osteoma (Plate 3). T h e lesion was curetted and culture of the curettings yielded S. schenckii. T h e symptoms settled with potassium iodide given over a period of 6 weeks. Case 3
A 5o-year-old man complained of pain over the lateral aspect of the proximal part of the left leg. T h e r e was no history of trauma. T h e lateral compartment was tender but minimally swollen. There were no skin lesions or enlarged lymph nodes and chest radiographs were normal. T h e ESR was 58 m m / h . Radiographs of the left leg revealed new bone formation of the proximal part of the fibula. Culture of biopsy material yielded S. schenckii. T h e patient received potassium iodide therapy for 5 weeks with a satisfactory outcome. Case 4
A 27-year-old farmhand presented with pain in the right buttock of 2 months' duration. T h e r e was no history of trauma but the patient was being treated for pulmonary tuberculosis. Clinically he was tender over the right ischium; rectal examination was normal. T h e sciatic nerve was intact. Chest radiographs revealed tuberculosis of the apex of the right lung. T h e ESR was 68 m m / h . T h e sputum was negative for acid-fast bacilli and fungal cultures were negative also. Curettings from the lesion over the ischium were caseous and yielded S. schenckii on cultures (Plate 4). Subsequent sputum examination was negative for fungal elements. T h e patient made an uneventful recovery after treatment with potassium iodide for 7 weeks. Discussion
Osseous sporotrichosis is a chronic infection of bone which may appear as an isolated lesion or part of disseminated infection. An extensive review of the American and English literature by Gladstone et al. (I97I) revealed only 22 patients with osseous sporotrichosis all of whom had positive cultures and
Journal of Infection
Plate I
Plate I. Well-defined circumscribed lytic lesion of the distal part of the left ulna.
S. GOVEND~R ET AL.
(Facing p. 274 )
Journal of Infection
Plate 2, Complete resolution of the lesion 6 months after surgery.
S. GOVENDER E T
AL.
Plate 2
Journal of Infection
Plate 3. Lytic lesion with a surrounding zone of dense sclerosis involving the left tibia.
S. GOVENDERE T
AL.
Plate 3
Journal of Infection
Plate 4
Plate 4. Lacto-phenol cotton blue preparation of culture showing hyphae with 'daisy head' appearance, x 400.
S. GOVENDER E T
AL.
Osseous sporotrichosis
275
bone involvement. Nineteen (86 %) of the 22 patients were male; 54% were farmers, labourers and gardeners. T h e patients ranged in age from 8 to 72 years, most being over the age of 40 years. T h e commonest bones involved were the tibia, fibula (eight patients), metacarpals and phalanges (six patients), radius and ulna (five patients). In I5 (68 %) patients local swelling developed over the site of bone destruction and in five cases there were signs of acute inflammation. Nine patients (4I %) had sinuses draining from the osseous lesions to the skin surface. Fourteen patients had distant skin lesions illustrating the disseminated nature of this disease. Seventeen patients had swelling of adjacent joints with limitation of movement. All the patients showed at least one local sign, i.e. swelling, a sinus tract a n d / o r inflammation of an adjacent joint. Thus, the combination of local areas of bone destruction and the appearance of distant skin lesions suggest the possibility of sporotrichosis although these signs are by no means pathognomonic. In ~9 of the 22 patients, the bony lesions were osteolytic with little reactive osteoblastic change. These radiographic findings are not specific for sporotrichotic osteomyelitis alone since similar changes may be seen in other fungal infections, in osseous tuberculosis, in metastatic carcinoma, in bone cysts, and in giant cell turnouts of bone. Only three of i2 patients made significant recovery after administration of potassium iodide. These three patients also had surgical debridement in addition to potassium iodide therapy. Surgical debridement was not done in the other cases for fear of causing permanent impairment of adjacent joint function since the lesions were juxta-articular. Six patients were treated with amphotericin B with excellent results, although amphotericin B produces histopathological evidence of persistent renal damage even though the renal function returns to normal 6 after cessation of treatment with the drug. Although potassium iodide has been shown to be ineffective in the treatment of osseous lesions 2'5'v we feel that thorough surgical debridement is an essential pre-requisite if the lesions are accessible as noted in our study. During surveillance ranging from 6 months to 2 years, our cases showed no evidence of recurrence and there was radiological evidence of healing. It has been noted that, in localised osseous fungal lesions, surgical debridement alone without antifungal agents results in a satisfactory outcome. 8'9 Potassium iodide is administered in a dosage of five drops 8 hourly in fruit juice or milk until a maximum dosage of 4o drops 8 hourly is attained. T h e therapy should be stopped and reinstituted with a smaller dose if there are side effects such as excess lacrimation, salivation and swelling of the salivary glands. Amphotericin B may also be used but it is important to monitor carefully the full blood count, renal and liver function and to start treatment with a small dosage. Itraconazole which is less toxic may be used for both superficial and deep sporotrichosis. (We thank Dr M. van Rensburg, Department of Microbiology, for culturing and identifying the organism, Mrs B. Katia for typing the manuscript and Mrs L. Roux, Department of Microbiology, for preparing the photomicrograph.)
276
S. GOVENDER E T AL.
References I. Lynch AC, Geraci JF, Young HH, Weed LA, Bahn RC. Systemic sporotrichosis with bilateral synovitis in the knees. Report of a case. Proc Mayo Clinic I963; 38: 358-366. 2. Kedes LH, Siemienski J, Braude AI. The syndrome of the alcoholic rose gardener. Sporotrichosis of the radial tendon sheath. Report of a case cured with amphotericin B. Ann Int Med I964; 6I: zI39-xI4I. 3. Wilson DE, M a n n JJ, Bennet JE, Utz JP. Clinical features of extra cutaneous sporotrichosis. Medicine i967; 46: 265-279. 4. Lurie HI. Five unusual cases of sporotrichosis from South Africa showing lesions of muscles, bones and viscera. Br J Surg I963 ; 5o: 585-59I. 5. Mikkelsen WM, Brandt RL, Harrel ER. Sporotrichosis : a report of Ie cases including two with skeletal involvement. Ann Int Med I957; 4I : 435-459. 6. Gladstone J, Littman M. Osseous sporotrichosis. Am J Med I97I ; 5I: I 2 I - I 3 3 . 7. Braude AI, Sporotrichosis. In: Braude AI, Davis CE, Fierer J, Eds. Medical microbiology and infectious disease. Philadelphia: WB Saunders, I98I: I573-I577. 8. Chelboun J, Nade S. Skeletal cryptococcosis. J Bone Joint Surg I977; 59:509-5 I4. 9. Govender S, Ganpath V, Charles RW, Cooper K. Localised osseous cryptococcal infection. Report of two cases. Acta Orthop Scan I988; 58: 720-722.
CASE REPORTS Osseous sporotrichosis S. G o v e n d e r , M. N. R a s o o l and M. N g c e l w a n e
Department of Orthopaedics, University of Natal Medical School, P.O. Box I7O39, Congella 4o13, Durban, South Africa Accepted for publication 6 May 1989 Summary We report on four patients with osteomyelitis due to Sporothrix schenckii who were successfully treated with potassium iodide. Introduction
Sporothrix schenckii was first isolated in I898 at Johns Hopkins Hospital by Schenck who cultured the fungus from a patient with multiple subcutaneous abscesses. 1 T h e fungus, which is saprophytic, has been found in the soil, shrubs, flowers and in timber. Most often, transmission to h u m a n beings arises through a breach in the skin, especially of the hands and feet during the handling of contaminated vegetation. T h e infection is commonly associated with farmers and gardeners and has been described as 'the syndrome of the alcoholic rose gardener'fl Cutaneous or lymphocutaneous lesions are the commonest manifestations of sporotrichosis. 3 After an incubation period varying from 7 to 40 weeks, the fungus produces a reddish purple nodule. A series of subcutaneous nodules develops along the path of the lymphatic drainage of the primary lesion. A few patients may develop deep-seated abscessesJ In the fixed cutaneous form, the process is restricted to the site of inoculation. T h e histopathological appearance of the nodule is that of a central core of caseous or purulent material surrounded by a zone of polymorphonuclear leucocytes and macrophages. Surrounding this central zone are sheets of epithelioid cells with an intermingling of multinuclear giant cells. Around the periphery, lymphocytes and connective tissue cells are the prominent featuresJ Individual lesions may vary by displaying some or all of the foregoing characteristics. T h e microscopical appearance is not specific for sporotrichosis and indeed cannot be differentiated from tuberculosis and other fungal lesions. Definitive diagnosis rests solely on culture of the organismJ T h e extracutaneous or systemic forms of the disease may follow the cutaneous lesions or arise in their absence. T h e y involve the lungs, musculoskeletal system 5 and other sites. Case reports Case x An 8-year-old boy presented with pain of 8 weeks' duration over the distal part of the left forearm. A lesion over the distal part of the left ulna had been o163-4453/89/o6o273 + o 4 $o2.oo/o
© 1989 The British Society for the Study of Infection 12-2
274
S. GOVENDER E T A L .
biopsied at a peripheral hospital. T h e biopsy had been reported as showing evidence of chronic non-specific infection. Clinically, the distal part of the forearm was tender over the ulna border. Apart from a well-healed biopsy scar there was no evidence of any cutaneous lesion or nodal involvement. Radiographs revealed a lytic lesion of the distal part of the ulna with periosteal new bone formation (Plate I). Apart from an ESR of 6o m m / h , all other haematological investigations were normal. Thorough curettage of the lesion yielded material from which S. schenckii grew in culture. T h e child was treated with potassium iodide for 4 weeks with complete resolution of thelesion (Plate 2). Case 2
A 29-year-old man presented with pain over the shin of the left leg of 4 months' duration. A year earlier he had fallen and sustained a minor abrasion over the tibia, which healed well. Clinically, he was tender over the tibia but without skin lesions. T h e ESR was 47 m m / h and a radiograph of the left tibia showed a lytic lesion with a well-defined zone of sclerosis. T h e appearance suggested a Brodie's abscess or an osteoid osteoma (Plate 3). T h e lesion was curetted and culture of the curettings yielded S. schenckii. T h e symptoms settled with potassium iodide given over a period of 6 weeks. Case 3
A 5o-year-old man complained of pain over the lateral aspect of the proximal part of the left leg. T h e r e was no history of trauma. T h e lateral compartment was tender but minimally swollen. There were no skin lesions or enlarged lymph nodes and chest radiographs were normal. T h e ESR was 58 m m / h . Radiographs of the left leg revealed new bone formation of the proximal part of the fibula. Culture of biopsy material yielded S. schenckii. T h e patient received potassium iodide therapy for 5 weeks with a satisfactory outcome. Case 4
A 27-year-old farmhand presented with pain in the right buttock of 2 months' duration. T h e r e was no history of trauma but the patient was being treated for pulmonary tuberculosis. Clinically he was tender over the right ischium; rectal examination was normal. T h e sciatic nerve was intact. Chest radiographs revealed tuberculosis of the apex of the right lung. T h e ESR was 68 m m / h . T h e sputum was negative for acid-fast bacilli and fungal cultures were negative also. Curettings from the lesion over the ischium were caseous and yielded S. schenckii on cultures (Plate 4). Subsequent sputum examination was negative for fungal elements. T h e patient made an uneventful recovery after treatment with potassium iodide for 7 weeks. Discussion
Osseous sporotrichosis is a chronic infection of bone which may appear as an isolated lesion or part of disseminated infection. An extensive review of the American and English literature by Gladstone et al. (I97I) revealed only 22 patients with osseous sporotrichosis all of whom had positive cultures and
Journal of Infection
Plate I
Plate I. Well-defined circumscribed lytic lesion of the distal part of the left ulna.
S. GOVEND~R ET AL.
(Facing p. 274 )
Journal of Infection
Plate 2, Complete resolution of the lesion 6 months after surgery.
S. GOVENDER E T
AL.
Plate 2
Journal of Infection
Plate 3. Lytic lesion with a surrounding zone of dense sclerosis involving the left tibia.
S. GOVENDERE T
AL.
Plate 3
Journal of Infection
Plate 4
Plate 4. Lacto-phenol cotton blue preparation of culture showing hyphae with 'daisy head' appearance, x 400.
S. GOVENDER E T
AL.
Osseous sporotrichosis
275
bone involvement. Nineteen (86 %) of the 22 patients were male; 54% were farmers, labourers and gardeners. T h e patients ranged in age from 8 to 72 years, most being over the age of 40 years. T h e commonest bones involved were the tibia, fibula (eight patients), metacarpals and phalanges (six patients), radius and ulna (five patients). In I5 (68 %) patients local swelling developed over the site of bone destruction and in five cases there were signs of acute inflammation. Nine patients (4I %) had sinuses draining from the osseous lesions to the skin surface. Fourteen patients had distant skin lesions illustrating the disseminated nature of this disease. Seventeen patients had swelling of adjacent joints with limitation of movement. All the patients showed at least one local sign, i.e. swelling, a sinus tract a n d / o r inflammation of an adjacent joint. Thus, the combination of local areas of bone destruction and the appearance of distant skin lesions suggest the possibility of sporotrichosis although these signs are by no means pathognomonic. In ~9 of the 22 patients, the bony lesions were osteolytic with little reactive osteoblastic change. These radiographic findings are not specific for sporotrichotic osteomyelitis alone since similar changes may be seen in other fungal infections, in osseous tuberculosis, in metastatic carcinoma, in bone cysts, and in giant cell turnouts of bone. Only three of i2 patients made significant recovery after administration of potassium iodide. These three patients also had surgical debridement in addition to potassium iodide therapy. Surgical debridement was not done in the other cases for fear of causing permanent impairment of adjacent joint function since the lesions were juxta-articular. Six patients were treated with amphotericin B with excellent results, although amphotericin B produces histopathological evidence of persistent renal damage even though the renal function returns to normal 6 after cessation of treatment with the drug. Although potassium iodide has been shown to be ineffective in the treatment of osseous lesions 2'5'v we feel that thorough surgical debridement is an essential pre-requisite if the lesions are accessible as noted in our study. During surveillance ranging from 6 months to 2 years, our cases showed no evidence of recurrence and there was radiological evidence of healing. It has been noted that, in localised osseous fungal lesions, surgical debridement alone without antifungal agents results in a satisfactory outcome. 8'9 Potassium iodide is administered in a dosage of five drops 8 hourly in fruit juice or milk until a maximum dosage of 4o drops 8 hourly is attained. T h e therapy should be stopped and reinstituted with a smaller dose if there are side effects such as excess lacrimation, salivation and swelling of the salivary glands. Amphotericin B may also be used but it is important to monitor carefully the full blood count, renal and liver function and to start treatment with a small dosage. Itraconazole which is less toxic may be used for both superficial and deep sporotrichosis. (We thank Dr M. van Rensburg, Department of Microbiology, for culturing and identifying the organism, Mrs B. Katia for typing the manuscript and Mrs L. Roux, Department of Microbiology, for preparing the photomicrograph.)
276
S. GOVENDER E T AL.
References I. Lynch AC, Geraci JF, Young HH, Weed LA, Bahn RC. Systemic sporotrichosis with bilateral synovitis in the knees. Report of a case. Proc Mayo Clinic I963; 38: 358-366. 2. Kedes LH, Siemienski J, Braude AI. The syndrome of the alcoholic rose gardener. Sporotrichosis of the radial tendon sheath. Report of a case cured with amphotericin B. Ann Int Med I964; 6I: zI39-xI4I. 3. Wilson DE, M a n n JJ, Bennet JE, Utz JP. Clinical features of extra cutaneous sporotrichosis. Medicine i967; 46: 265-279. 4. Lurie HI. Five unusual cases of sporotrichosis from South Africa showing lesions of muscles, bones and viscera. Br J Surg I963 ; 5o: 585-59I. 5. Mikkelsen WM, Brandt RL, Harrel ER. Sporotrichosis : a report of Ie cases including two with skeletal involvement. Ann Int Med I957; 4I : 435-459. 6. Gladstone J, Littman M. Osseous sporotrichosis. Am J Med I97I ; 5I: I 2 I - I 3 3 . 7. Braude AI, Sporotrichosis. In: Braude AI, Davis CE, Fierer J, Eds. Medical microbiology and infectious disease. Philadelphia: WB Saunders, I98I: I573-I577. 8. Chelboun J, Nade S. Skeletal cryptococcosis. J Bone Joint Surg I977; 59:509-5 I4. 9. Govender S, Ganpath V, Charles RW, Cooper K. Localised osseous cryptococcal infection. Report of two cases. Acta Orthop Scan I988; 58: 720-722.