I Polyarticular Arthritis Due to Sporothrix Schenckii I Polyartikulare Arthritis Durch Sporothrix Schenkii Marci Lesperance, D

mycoses 31, No. 12 (1988) Sporothrix Arthritis 599

mycoses 31 (12) 599-603 - accepted/angenommen: October 17,1988 0 Grosse Verlag Berlin 1988 I Polyarticular Arthritis Due to Sporothrix schenckii I Polyartikulare Arthritis durch Sporothrix schenkii Marci Lesperance, D. Baumgartner and Carol A. Kauffman Division of Infectious Diseases, Department of Internal Medicine, University of Michigan Medical School and Veterans Administration Medical Center, Ann Arbor, Michigan, U.S.A.

Key words: Sporothrix schenkii -fungal arthritis - systemic sprotrichosis - imidazoles Schliisselworter: Sporothrix schenkii - pilzbedingte Arthritis - systemische Sporotrichose - Imidazole

Summary: Sporothrir schenckii infection gen des langen Zeitraums vom Einsetzen usually presents as cutaneous or lympho- der Symptome bis zur Diagnose. cutaneous disease. Rarely, this dimorphic fungus causes isolated osteoarticular infection. The patient described herein had Introduction polyarticular sporotrichosis with contiguous osteomyelitis presenting as carpal tun- Sporothrix schenkii is a dimorphic fungus nel syndrome 5 years previously. He re- which commonly causes cutaneous or lapsed after 2 g of amphotericin B but re- lymphocutaneous infection following di- sponded to itraconazole, a new oral anti- rect inoculation through the skin (21). Ex- fungal agent. However, he was left with tracutaneous disease is much less common severe limitation of motion in the affected and may include osseous and joint invol- joints due to the long interval from onset vement, as well as lung, genitourinary, and of symptoms to diagnosis. central nervous system infection (25). Rarely, sporotrichosis presents as isolated Zusammenfassung: Die Sporothrix joint infection without an obvious primary schenckii-Infektion ist gewohnlich an der lesion elsewhere (2, 4, 12, 16). Given the Haut oder im Bereich der Haut- rarity of articular sporotrichosis, the diag- Lymphknoten lokalisiert. Selten verur- nosis is often not made until the disease is sacht dieser dimorphe Pilz eine isolierte far advanced. We report a patient with in- osteoartikulare Infektion. Der hier vorge- dolent polyarticular sporotrichosis of five stellte Patient hatte eine polyartikulare years duration, in whom progressive de- Sporotrichose mit benachbarter Osteo- struction of several joints occurred before myelitis, was sich 5 Jahre zuvor als Kar- the diagnosis was finally established. paltunnel-Syndrom dargestellt hatte. Der Patient erlitt nach Verabreichung von ins- gesamt 2 g Amphotericin B einen Ruck- Case Report fall, sprach jedoch auf Itraconazol an. Es verblieben aber schwere Bewegungsein- A 34 year old man presented to another schrankungen der befallenen Gelenke we- hospital in 1981 with swelling of the right 600 Marci Lesperance et al. rnycoses 31, No. 12 (1988) wrist and numbness and tingling of the fin- vealed necrotic granulomatous inflamma- gers. He underwent carpal tunnel release tion, but no fungi were seen. without relief of his symptoms. Over the The patient was begun on oral flucona- next several years he had swelling and ery- zole, 50 mg daily, but he was noncom- thema in the right forearm and progressive pliant with his medicines and began drink- limitation of range of motion and pain in ing heavily, necessitating hospital ad- the right elbow, necessitating retirement mission 2 weeks later. He was then treated from construction work. In 1985 he de- with intravenous amphotericin B, receiv- veloped pain, swelling, and erythema of ing a total of 2 g. He continued to have the left wrist, left elbow, and left knee. slight swelling of the left olecranon bursa In January 1986 he presented to the and pain in both elbows; however, the left Ann Arbor Veterans Administration knee no longer was swollen or painful. Medical Center where he was found to He did well until September 1987 when have decreased range of motion in the he noted increasing pain in both elbows right elbow, a small effusion in the left and marked limitation of movement in the knee, and swelling of the left olecranon left elbow. He felt fatigued but had no bursa. Past history was significant only for other complaints. Roentgenographic alcoholism and hypertension. Rheumatoid evaluation showed destruction of the left factor and anti-nuclear antibody tests elbow joint (Fig. 1). An open biopsy of the were negative, the Westergren sedimenta- left elbow and bursectomy were per- tion rate was 20 mm/h, and he was found formed. Examination of the tissue re- to be HLA B-27 positive. Aspirate of sy- vealed granulomatous inflammation and novial fluid from the right elbow revealed several budding yeasts. Culture of bone, as 14 000 white blood cells/mm3 with 74 o/o well as synovial fluid from the left elbow, lymphocytes. Culture of the aspirate was again yielded S. schenckii. The patient was negative for bacteria, fungi, and acid-fast begun on 400 mg itraconazole orally daily bacilli. He was thought to have seronega- and underwent further debridement of the tive rheumatoid arthritis and was treated joint. After 1 month S. schenckii was no with high dose aspirin with only minimal longer recovered from the drainage, and improvement. after 3 months, the wound had healed Over the next six months he continued completely. He was treated for a total of to have swelling, pain, and limitation of six months at which point he again began movement in both elbows and the left drinking and again became non-compliant knee. Examination in September 1986 with his drug regimen. Although the pain showed effusions in both elbows and the had decreased, he remained with limita- left knee, marked swelling of the left ole- tion of movement in both.elbows. cranon bursa, and severe limitation of movement of the right elbow. Roentgeno- grams of the right elbow showed destruc- Discussion tion of the joint and osteomyelitis of the proximal ulna and radius. Synovial fluid Sporothrix schenckii is ubiquitous in na- from the left elbow revealed 2800 white ture, occurring in many different climates blood cells/mm3, 75 '/O of which were and ecosystems (21). The majority of lymphocytes. Biopsy of the right proximal cases involve patients with outdoor occu- 1 ulna and radius was performed, and cul- pations or avocations, such as nursery tures from these specimens, as well as bur- workers, construction workers, farmers, sal fluid from the left olecranon bursa and miners, and gardeners (22). It has been joint fluid from the left knee, all yielded S. suggested that the preponderance of cases schenckii. Examination of the tissue re- in men (75 %-go o/o of cases) may be re- mycoses 31, No. 12 (1988) Sporothrix Arthritis 601

Fig. 1: Radiograph of the left elbow showing extensive demineralization and destruction of the joint due to Sporothrix schenckii.

lated to occupational exposure rather than with his ability to take his medications any intrinsic male susceptibility. properly. The vast majority of cases of sporotri- The pathogenesis of osteoarticular spo- chosis manifest only cutaneous or lympho- rotrichosis is unclear. While some cases cutaneous involvement (21). Osseous and are clearly associated with traumatic ino- articular infections are uncommon and are culation, these are in the minority. Cuta- often associated with underlying diseases, neous disease may be associated with con- such as diabetes mellitus, hematologic ma- tiguous bone and joint involvement, but lignancies, and alcoholism (2, 13, 18, 25). this is quite rare. It is most probable that The association of alcoholism with serious the mode of infection is via hematogenous infections with sporotrichosis is particu- dissemination after initial pulmonary in- larly strong. No explanation for this asso- volvement, as occurs with other deep fun- ciation has been found other than the in- gal infections. creased likelihood of traumatic inocula- Many cases of sporotrichal osteoarticu- tion of the organism; however, this expla- lar disease have no associated cutaneous nation is inadequate to explain the pro- lesions. The disease affects the knee most pensity for disseminated disease, espe- often, followed by involvement of the cially in a patient without a local cuta- small joints of the hands and wrists, the neous lesion (2, 4, 10, 12, 16). Alcohol- ankles, and elbows (2). Monoarticular and ism appeared to play an important role in polyarticular involvement occur equally our patient’s disease and also interfered frequently. Tendon sheaths and bursae 602 Marci Lesperance et al. mycoses 31, No. 12 (1988) may be infected, with the popliteal and The most useful method to diagnose olecranon bursae involved most com- sporotrichosis is culture of the organism monly. Isolated tenosynovitis, presenting from bone, synovial tissue, or joint fluid. as carpal tunnel syndrome, as noted in- Frequently, synovial fluid will yield Sporo- itially in our patient, has been described thrix on culture, in contrast to other fungal (24). arthritides, such as coccidioidomycosis,in Sporotrichal articular disease is mani- which synovial fluid is rarely positive. fested by joint pain, swelling, stiffness, ery- Skin testing and serology have not been thema and limitation of range of motion. useful because of a high incidence of posi- Low grade fever is sometimes present but tive skin tests and the lack of a sensitive there are usually no other constitutional and specific antibody assay for S. schenckii. symptoms. Frequently, as in our patient, Newer serologic techniques, including a infection is not thought of, fungi are not latex agglutination test and an enzyme im- specifically sought, and the infection pro- munoassay, appear to be useful, but will gresses slowly over years. Arthrocuta- have to be studied in more patients (23). neous fistulae, parasynovial abscesses and The differential diagnosis of osteoar- contiguous osseous involvement may ticular sporotrichosis includes rheumatoid occur, usually later in the course (2). arthritis, gout, sarcoidosis, pigmented vil- Laboratory studies are not very helpful. lonodular synovitis, and tuberculous arth- The sedimentation rate may be elevated or ritis, as well as arthritis due to other fungi normal, as in our patient. Leukocytosis is - blastomycosis, histoplasmosis and coc- uncommon. In the few cases reported, sy- cidioidomycosis (5, 12, 14, 16, 20). Espe- novial fluid showed from 2800WBC/ cially with indolent polyarticular involve- mm3 to 60 000 WBC/mm3 (1, 3, 7, 8, 11, ment, fungal infection is not considered 12, 13, 15, 16, 26). Neutrophils usually and a diagnosis of rheumatoid arthritis is predominate (3, 11, 12, 15, 16) although made (5,16,20). our patient and several others had mostly The morbidity due to sporotrichal os- lymphocytes (1, 8, 26). Generally, the sy- teoarticular disease may be severe. The in- novial fluid glucose has been low when fection is often not diagnosed until perma- measured (3,16). Only one case report de- nent joint damage occurs, as was the case scribes Sporothrix seen on Gram’s stain of in our patient. In Bayer’s review of 44 synovial fluid (7). cases, the mean interval from onset of Radiographic examination most com- symptoms to diagnosis was 17 months . monly shows osteoporosis of the contigu- (with a range from 2 months to 8 years) ous bony surfaces of affected joints, with (2). Difficulties leading to a delay in diag- juxtaarticular cartilage erosion and soft nosis include low clinical suspicion in the tissue and parasynovial swelling. Discrete absence of cutaneous disease, the nonspe- punched out bony lesions may also be cific nature of the inflammation noted on seen. On histopathologic examination, biopsy, the paucity of organisms seen in sporotrichal arthritis usually shows a joint fluid or histologic examination, and chronic noncaseating granulomatous sy- the failure to ask for culture for fungus. novitis that is similar to that seen in rheu- In contrast to cutaneous and lymphocu- matoid arthritis (24). Occasionally, casea- taneous sporotrichosis, which can be tion may occur mimicking tuberculous cured with iodides, osseous and articular arthritis. There are usually very few organ- sporotrichosis are not responsive to isms noted in the synovium or bone, mak- iodides (4, 10, 17). The best results have ing it difficult to identlfy S. schenckii, even been obtained with amphotericin B, with with careful serial sectioning and special or without surgical debridement (17). At stains. least 1 g of amphotericin B appears to be mycoses 31, No. 12 (1988) Sporothrix Arthritis 603 necessary for cure; however, some pa- the bones and joints with ketoconazole. J. Infect. tients, including the current case, relapsed Dis., 147, 1064-1069. 10. Kedes, L. H., J. Siemienski, A. I. Braude (1964): with even greater amounts of ampho- The syndrome of the alcoholic rose gardener: tericin B (3). Sporotrichosis of the radial tendon sheath, re- Ketoconazole has been very disappoint- port of a case cured with amphotericin B. Ann. Intern. Med., 61,1139-1141. ing in treating sporotrichosis (6, 9, 18). 11. Kahn. M. I., A. Goss, A. Gotsman. M. S. Asvat Recently, interest has centered on the use (1983): Sporotrichosis arthritis. S: A. Med. J., of the newer oral triazole compound, itra- 64,1099-1101. 12. Levinskv. W. J. 11972): Soorotrichial arthritis: conazole. In vitro studies show that S. Report b;f a case'mimihciig gout. Arch. Intern. schenckii is inhibited by itraconazole, and Med., 129,118-119. preliminary evidence suggests this agent is 13. Lynch, P. J., J. J. Voorhees and E. R. Harrell (1970): Systemic sporotrichosis. Ann. Intern. useful in the treatment of cutaneous and Med., 73,23-30. lymphocutaneous sporotrichosis (19). Our 14. Marrocco, G. R., W. S. Tihen, C. P. Goodnough, patient responded well to itraconazole, R. J. Johnson (1975): Granulomatous s novitis and osteitis caused by Sporothrix schenclii. Am. showing eradication of the organism and J. Clin. Pathol., 64,345-350. healing of the surgical wound. However, 15. Mikkelsen, S. M., R. L. Brandt, E. R. Harrell he was left with significant loss of function (1957): Sprotrichosis: A report of 12 cases, including two with skeletal involvement. AM. 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