DISSEMINATED RHINOSPORIDIOSIS

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Barik B. K., Padhan P.

Abstract:

A rare case of disseminated rhinosporidiosis is reported in a young male patient. The pathophysiology, clinical feature, diagnosis and management of this condition are reviewed.

Key words: Rhinosporidiosis, Rhinosporidium Seeberi

Indroduction:

             Rhinospordiosis is a chronic granulomatous disease characterized by production of polyps or other manifestations  of hyperplasia on mucous membrane surfaces, the etiologic agent being Rhinosporidium seeberi. The disease was First describe d by seeber (1900) in Argentina. This noval pathogen commonly affects mucosa of nose, eye and upper aero-digestive tract of men and animals. Isolated deep seated Rhinosporidiosis is rare. Diagnosis is mainly based on clinical suspicion and histopathological confirmation. At present, no existing medical treatment cures the disease and  excision of the mass with cauterization of the base is considered as treatment of choice.

Case Report

            A 35 year old male patient came to us with history of multiple swellings all over the body (Fig-1) for last one year, distension of the abdomen for last 2 months and loss of appetite for last 20 days.

            The patient came to us for loss of appetite. He was not a known case of DM, HTN, SCD, Pul TB. On examination, the patient was chchectic with mild pallor, noi icterus, with a pulse rate of 84/minute, regular, BP-100/80 mmHg,  CVS-NAD, Chest:-NAD, Abdomen: No hepatosplenomegaly or engorged abdominal veins. There were multiple subcutaneous swellings ranging from size 1  1cm to 5  7cm.

Investigations:

 Hb%-8.0gm%

TLC:-6,000/mm

DC:-N-82%, L-17%, E-01%

FBS:-106 mg%

ESR:- 5mm in 1^st hr,

Sr. Urea –30 mg%

Sr. Creatinine :- 1.43 mg%

Urine – NAD

HbsAg –Negative

Anti-HCV:- Negative     

ELISA for HIV- Negative

S.Bilirubin – 0.6 mg%

SGPT :-51 IV/It.

SGOT :-41 lU/Lt

Sr.Alk. phosphatase :-221 lU/Lt

Sr. Albumin: 2.9 gm%

USG of Abdomen:- Normal

Chest X-Ray PA view –Normal

            Biopsy study of subcutaneous swelling: Large numbers of spherules (size –10-200 um) in a stroma of connective tissue and capillaries. The spherules contain thousands of endospores.

            The patient was diagnosed as a case of disseminated rhinosporidiosis. The patient was stared with tab Dapsone 100 mg/day & Vit-B complex. On regular follow up since last  6 months, the size of subcutaneous swelling were regressing.

Discussion :

Definition:

            RHINOSPORIDIOSIS is a chronic granulomatous disease characterized  by production of polyps or other manifestations of hyperplasia on mucous membrane surfaces. The etiologic agent is Rhinosporidium seeberi.

Historical review:

            Most of the early studies of rhinosporidiosis were made in India and Ceylon where the disease occurs frequently. Sporadic case have been detected and studied in many parts of the world. The  systematic position of R. seeberi is still uncertain. Most  investigators consider it has not been isolated in culture.

 Clinical Types:

            Friable, highly vascular, sessile or pedunculated polyps may appear on almost any mucosal  surface, and rarely secondary lesions are found on skin, probably as aresult of autoinoculation by scratching. Lesions of the mucosae often spread by extension beyond the mucocutaneous border.

            Primary lesions appear most often on the nasal mucosa and are accompanied by painless itching and a profuse mucoid discharge. The lesion is at first flat and sessile. Later hyperplastic growth greatly exceeds lateral extension of the lesion so that a polypoid mass much larger than the peduncle develops. The polyp may extend from the neres into the pharynx or externally over the lip and may reach weight of 20grams. It is friable and bleeds freely after trauma. Its surface is mucoid and papillate or so lobulate that its surface suggests that of a cauliflower. The color varies from pink to purplish red, and close examination of thesurface mayh reveal minute white sports which are the mature sporangia of the fungus. Lesions are found also on the larynx, Penis vagina, rectum and skin.

            Lesions of the eye may cause symptoms similar to those produced by a foreign body, lacrimation or photophobia. Growth of the polyp may cause eversion of the lid. Lesions on th eskin being as papillomas and become warty with inclusions of myxomatous material. They are relatively painless except when on the sole of the foot and when they become so large as to be uncomfortably heavy. Dissemination to visceral organs is rare.

Differential diagnosis:

            Typical lesions of rhinospordiosis can be recognized usually by the pink to purple colour, friable consistency and the presence of barely visible white sporangia within the polyp. Atypical lesions or those in unusual anatomical sites must be differential from warts, condylomata and hemorrhoids.

Immunology:

            Little is known about the immunology of rhinosporidiosis.

Pathology:

            On the examination of the gross tissue, unless rhinosporidiosis has been suggested by the clinician, or by the history of the patient’s geographic residence, the pathologist may consider the specimen an ordinary nasal polyp. The correct diagnosis can usually be made without difficulty on examination of routine H and E stained slide. Under the scanning lens of the microscope, although the polypoid structure may be evident, the histopathologic pattern differs greatly from tht of the common nasal polyp. The most striking feature is the presence in the stroma or epidermis of numerous sharply defined globular cysts which usually vary from 10 to 200 m in diameter. Some of the cysts may be partly collapsed, assuming a semilunar shape. In contrast to the loose, edematous, myxomatous stroma of the ordinary nasal polyp, the stroma in rhinospordiosis is rather dense. There is a chronic inflammatory reaction in which neutrophils, plasma cells and lymphocytes are prominent. In contrast to the usual nasal polyp. Eosinophils are inconspicuous. Occasionally purulent microabscesses occur.

            The cysts of all size have a sharply defined chitinous appearing wall. In a large maturing cyst the wall alone may be 5 m thick. Histologically, rhinosporidiosis should be differentiated, specially in immuno-suppressed persons with other fungal  infection like Coccidioides immitis.

Epidemiology:

            Differential incidence: Although rhinosporidiosis is seen most often in children and young adults, it occurs at any age. No racial difference in susceptibility are recognized. The disease is seen much more frequently in men than in women, but the extent to which this difference is related to greater frequency or severity of exposure is difficult to evaluate. Infections are seen most often in labourers and in those with frequent exposure to water of streams and pools.

Geographic distribution:

            Rhinosporidiosis is found must often in India and Ceylon, but it is reported also from the East Indies, the Malay, States, the Philippines, Iran, South Africa, Italy, England, Scotland, Southern United States, Mexico, Cuba, Argentina, Brazil and Paraguay.

Source of infection:

            The disease is not contagious, and sources of infection are exogenous. The frequent history of prior extended to water of pools and rivers and the occurance of multiple cases among those members of a group of workmen most intimately and repeatedly exposed to water source suggest the R. seeberi has a natural habitat in water. Rhinosporidiosis was observed in workmen who dived under water to bring up san din buckets, but not in their associates who carried the sand from the water’s edge. It has been suggested that water insects or fish may be hosts of the fungus.

Laboratory diagnosis:

            Direct examination of the surface of the polyp may reveal the subsurface position of sporangia which are white and so large (up to 350 m in diameter) that thery can be seen with naked eye. Dissection of sporangia or excision and microscopic examination of tissue confirms the diagnosis. Culture is not successful, and the inability of R. seeberi to grow on artificial media, as well as some peculiarties about its reproductive cycle in tissues, have raised the question whether it is actually a fungus.

            It is resembles in general appearance and in manner of sporulation some species of synchytrium, which are obligate parasites of plants, and which produce characteristic galls on the host plant. Animal inocultion is not helpful in diagnosis. Although R.seeberi is found in natural infections of horses, mules and cows, experimental infections usually do not succeed. Recently molecular methods like polymerase chain reaction are being developed for diagnosis.

Conclusion:

            Rhinosporidiosis should be suspected or considered in all cases of swellings of nose, nasopharynx and skin. Although disseminated Rhinosporidiosis is very rare, still remains a possibility and requires a different mode of treatment. Presently the medical treatment of Rhinosporidiosis is not satisfactory and requires further study and research.

References:

1.                  Caldwell, G.T. and Roberts, J.D.: Rhinosporidiosis in the United States J.A.M.A. 1938; 110,1964.

2.                  Karunaratne W.A.E.: Rhinosporidiosis in Man, London, Athlone Press, 1964.

3.                  Weller, C.V. and Riker, A.D.: Rhinosporidiosis in Man, London, Athlone Press, 1964.

4.                  Weller, C.V. and Riker, A.D.: Rhinosporidiosis seeberi Am.J.Path, 1930,6,721-732.

5.                  Baron, E.J., Peterson, L.R. Finegold, S.M. New, Controversial difficult-to-cultivate or non-cultivate etiological agents of disease in Bailey and Scott’s Diagnostic Microbiology, 9^th Edition, Mosby, st.Louis, Baltimore, Boston, 1994; p-585.

6.                  Gori, S. Scasso, A.: Cytologic and differential diagnosis of rhinosporidiosis: Acta Cytologica, 1994: 38(3):361-366.

7.                  Saha, S.NB., Mondal, A.R. Bera, S.P., Das S., and banerjee, A.R.: Rhinosporidial infection in West Bengal – Calcutta based hospital study. Indian Journal of Optolaryngology & Head Neck Surgery – 2001:53(2):100-104

Leptospirosis with Jarisch-Herxheimer reaction – A case report

Barik B.K., Padhan P.

Abstract:

            We report a reare case of leptospirosis who developed Jurish – Herxheimer reaction after initiation of treatment. The mechanism of this reaction and its pathophysiology are reviewed.

Key words: Jarisch-Herxheimer, Leptospirosis

Introduction:

Lleptospirosis is caused by Leptospira interrogans (e.g. serogroup L. icterohaemorrhagiae). Spread is bny contact with infected rat urine, e.g. while swimming, taking pond bath. They usually present with fever, jaundice, headache, red conjunctivae, tender legs (myositis), purpura, hemoptysis, hematemesis, or any bleeding, Meningitis, myocarditis and renal failure may develop.

Case Report:

            A 32 year old male presented in August 2002, with a 7day history of fever, headache, myalgia and nausea. He had always been fit and well. On examination he was hemodynamically stable, pyrexia, and had meningism, and mild abdominal tenderness, without hepatosplenomegaly. Blood tests showed 9.6 x 10⁹/L neutrophils, 125 x 10⁹/L platelets, but no other abnormalities. We started intravenous cefuoxime 1.5gm twice daily and oral doxycycline 100mg twice daily, to cover meningococal, streptococcal, salmonella and other gram negative and typical pathogens. Blood tests 12 hr. later after admission showed urea and creatinine normal aST 110/U/Lt, ALT 176 U/L. Tests for malaria parasite was negative, widal test negative abdominal sonogram revealed no abnormality. Blood for culture and sensitivity was sent.

            The patient became increasingly breathless and hypoxaemic. A chest rediograph showed mild cardiomegaly and an electrocardiograph (ECG) showed low voltage with widerspread ST segment changes. A transthoracic echocardiogram showed mold dilation of left ventricle. Pre-existing cardiac or pulmonary disease was considered unlikely in this otherwise healthy male. The patient was started on intravenous dexamethasone, dopamine infusion and oxygen inhalation. His condition improved over next 48 hours. The report ECG and transthroacic echocardiogramon day 5 showed complete resolution of previous abnormalities. He was discharged after finishing a week’s course of the antibiotics, and soon returned to work. He is doing well since then when last seen in Dec. 2002.

            We subsequently diagnosed acute leptospirosis by a leptospira spp. 1gM antibody titre of 1:2560, from blood sample taken on day 8 of admission (negative on the first day of hospitalization). The patient’s acute deterioration after antibiotic treatment was probably due to a Jarisch-Herxhemier reaction.

Discussion:

            Cardiovascular involvement can occur in both the septicaemic and immune phases of Leptospira infection. Minor ECG changes and pulmonary edema have been reported in the first week of illness, but cardiomegaly is unusual.

            The Jarisch Herxheimer reaction is a recognized complication of  antimicrobial treatment of spirochaetal infections. Lysed bacteria releasel LPS, triggering release of cytokines including tumour necrosis factor-a and iL-6 & 8. Antibiotics, including tetracyclines, b-lactams and cephalosporins trigger LPS release from bacteria. LPS induces NO mediated vasodilation resulting in systemic hypotension. LPS can also induce pulmonary hypertension.

Conclusion:

            Leptospirosis is relatively uncommon condition in this part of Orissa; Milder cases are underdiagnosed.

            It is not possible to predict which patients might develop a Jarisch Herxhemier reaction after starting treatment. Physicians should know that this reaction can include systemic gypotension. And acute pulmonary edema and that most patients recover fully with appropriate supportive therapy.

References:

1.                  Wesely Farr R. leptospirosis, Clin. Infect Dis. 1995; 21:18

2.                  Friedland JS, Warrell DA. The Jarisch Herxhemier reaction in leptospirosis: possible pathogenesis and review. Rev. Infect. Dis. 1991;13:207-10.

3.                  Negussie Y, Remick DG, Deforge LE, Kunkel SL, Eynon A, Griffin GE, Detection of plasma tunour necrosis factor, interleukins 6, and 8 during the Jarisch Herxhemier reaction of relapsing fever J. Exp. Med. 1922;175;1207-12.

4.                  Evans ME, Pollack M. Effect of antibiotic class and concentration of the release of lipopolysaccharide from Escherichia coli. J. Infect. Dis. 1994; 169:471-73.

Plasmacytoma, A relatively uncommon presentation

Barik B.K., Pusparani Das., Sagrika  Tripathy, Sethy R.C., Bhakta S., Padhan P.

Abstract:  We report a case of plasmacytoma in a middle – aged man who subsequently developed multiple myeloma. The pathophysiology, diagnosis and treatment of this condition are briefly reviewed.

Introduction:

      Plasma cell disorders are either generalized or localized. Generalized plasma cell proliferation with ostelytic bone lesion and monoclonal component of protein in serum electorphoresis is commonly known as multiple myeloma (M.M.). But 10% of all plasma cell disorders can present as localized plasmacytoma. Plasmacytoma may be two types:

1.                  Solitary osseous plasmacytoma (SOP)

2.                  Extramedullary plasmacytoma (EMP)

Usually they present at the age of 50 years, about 20yr before the average of presentation of multiple myeloma². Though majority of SOP and about 50% EMP can progress to M.M. in long course, but their early detection is very important for the treatment point of view. Local radio therapy with or without chemotherapy can cure these patients.

Case Report:

A 55 yr male was admitted on 25.06.03 with the complaints of swelling over sternal  region, noted for last 6 months. It was gradually increasing in size and became painful for last 4 months. He was also complaining of left shoulder pain. For last few days low back pain also started. This pain was nagging in character and increased with movement. His urine output normal, mild wt loss was also associated with it he was mildly anemic and his cardiovascular; CNS and respiratory system had no abnormality. Hepatosplenomegally was absent. His BP was 120/80 and pulse 80/min.

Sternal swelling was firm, mildly tender, nonpulsatile, measuring = 5cm x 9cm x 1.2cm.

On the day of admission FNAC of swelling was done and it reveled plasmacytoma. Serum and urine were sent for M band and Bence Jonce proteins respectively, but both of them were negative. But skull X-ray and Chest X-ray revealed punch out lesions and bone marrow from Rt iliac crest detected plasma cell infiltration (>85%). Hb% was 9.6gm. Urea 18mg% creatinine 1.2mg%. liver function test normal and sedimentation rate 60mm in 1^st hr.

Two days after admission, patient fell down in bathroom and the shaft of the humerus and femur were fractured. Plaster cast was done on next day.

Five days after admission, chemotherapy was started with cyclophosphamide 200mg/m² and predinisolone 80mg/m² for 54 days along with supportive therapy.

            Except mild pain abdomen and chest pain no other complication developed. On follow up after 4 weeks, swellin gover sternum regressed and sternal depression developed at that place due to destruction of mass along withribs instability. No radiotherapy was given.

Discussion

            Solitary plasmacytoma is comparatively an uncommon entity. When the above mentioned patient presented with sternal swelling it gives the suspicion of mediastinal pathology. But firm nature and ultimate FNAC report disclosed it as a plasmacytoma. Early age of presentation (55yrs) and few days H/O of low back pain also point out towards psasmacytoma. But as every patient with plasmacytoma should be a suspected of multiple myeloma, B.M. examination and X-ray skull were done. This led to the diagnosis of the patient as a case of MM. M band negativity in this case tells about non –secretory nature of the tumor,which is more aggressive. This can explain why a patient develops pathological fracture so early. b₂ Micro globulin can indicate about the prognosis of the patients, but not in this case due to lack of facility. As this case converted to M.M⁶, systemic chemotherapy was given instead of local irradiation.

            This is a case of plasmacytoma,is rare, it should be considered for any abnormal bony shelling and they should be further followed up to know their progression to multiple myeloma.

REFERENCE

1.      Meis J.M et al- Solitary plasmacytoma of bone and extramedullary plasma – cytoma: Aclinicopathological and immunological study –Cancer 1987 , 5:1475 –1785.

2.      Alexamin R. Localized and indolent myeloma 1980;56:521-526.

3.      Dimoponlos MA et al- Curability of slitary bone plasmacytome. J.Clinical Oncology. 1992, 10:587-590.

4.      Mouloponlos La et al – MRI in staging of solitary plasmasytoma of bones. J. of cli. Onco. 1993:11:1311-1315.

5.      Aviles Aet al – Serum b₂ microglobulin in solitary plasmacytoma- Blood 1990: 76 : 1663.