Case Series Rhinofacial Entomophthoramycosis
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SOUTHEAST ASIAN J TROP MED PUBLIC HEALTH CASE SERIES RHINOFACIAL ENTOMOPHTHORAMYCOSIS; A CASE SERIES AND REVIEW OF THE LITERATURE Juvady Leopairut1, Noppadol Larbcharoensub1, Wichit Cheewaruangroj2, Somnuek Sungkanuparph3 and Boonmee Sathapatayavongs3 1Department of Pathology, 2Department of Otolaryngology, 3Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand Abstract. Rhinofacial entomophthoramycosis is an uncommon chronic mycotic disease caused by exposure to the organism Conidiobolus coronatus. The authors report a case series of 5 patients with rhinofacial entomophthoramycosis and re- view the literature. All patients had typical involvement of the rhinofacial area with formation of subcutaneous lesions causing a chronic granulomatous inflam- matory response with tissue eosinophilia and Splendore-Hoeppli reaction. Diag- noses were made based on histopathologic examination in all cases and fungi were isolated and identified in one case. The clinicopathologic features and therapeutic management of rhinofacial entomophthoramycosis are described. Key words: entomophthoramycosis, phycomycosis, zygomycosis, Conidiobolus spp, rhinofacial INTRODUCTION 2004; Sugar, 2005; Chayakulkeeree et al, 2006). Entomophthoramycosis conidio- Rhinofacial entomophthoramycosis is balae occurs predominantly in immuno- an uncommon chronic mycotic disease in competent patients, and involves the head humans occuring in tropical and subtropi- and face. The disease is characterized by cal regions of the world. The term rhino- slowly progressive swelling of the soft tis- facial entomophthoramycosis encom- sue of the rhinofacial area and is occasion- passes various diseases caused by fungi ally mistaken for malignancy or tubercu- of the order Entomophthorales and class losis in clinical practice. Histopathologic Zygomycetes (Phycomycetes). The Ento- features reveal broad mycelial filaments mophthorales has two genera, Conidio- with infrequently septation surround by bolus and Basidiobolus, producing rhino- a Splendore-Hoeppli reaction. facial and subcutaneous entomophtho- The authors describe here five cases ramycosis, respectively (Prabhu and Patel, of rhinofacial entomophthoramycosis in Correspondence: Noppadol Larbcharoensub, immunocompetent patients, diagnosed Department of Pathology, Ramathibodi Hos- over a 15-year period on the basis of the pital, Mahidol University, 270 Rama VI Road, characteristic morphology of fungal ele- Ratchathewi, Bangkok 10400, Thailand. ments in infected tissue and on histopatho- Tel: +66 (0) 2354 7277; Fax: +66 (0) 2354 7266 logic findings. In one case reported, tissue E-mail: [email protected] culture revealed Conidiobolus coronatus. 928 Vol 41 No. 4 July 2010 RHINOFACIAL ENTOMOPHTHORAMYCOSIS CASE SERIES was noted. A culture of the right inferior Case 1 turbinate biopsy recovered Conidiobolus coronatus. The mass was completely re- A 44-year-old Thai male teacher liv- moved and a right maxillectomy was per- ing in Saraburi Province, Thailand, first formed. Medical therapy included a com- came to Ramathibodi Hospital in January bination of a saturated solution of potas- 1996, complaining of a firm mass on the sium iodide (SSKI) (40mg/kg/day) and right side of the nose with gradually in- trimethoprim/sulfamethoxazole. The treat- creasing nasal obstruction of one-year du- ment was continued for about 12 weeks. ration. There was no history of trauma or The patient was followed up in the Ear- insect bites. Clinical examination revealed Nose-Throat clinic for 14 years without any the patient was in good health. Physical evidence of recurrence. examination showed a firm mass on the inferior turbinate expanding to the exter- Case 2 nal opening of the right nose. The first bi- A 20-year-old healthy Thai male opsy revealed granulation tissue. Rhinos- came to Saraburi Hospital in September copy disclosed an infiltrative mass of the 1999 with a complaint of rapidly progres- lateral part of the nose compressing the sive left nasal swelling, starting 3 months right inferior turbinate, causing obstruc- prior to admission. There was no history tion of the right maxillary and right ante- of nasal discharge, epistaxis, trauma or rior ethmoid openings. The mass was firm evidence of insect bites. He had a biopsy with contact bleeding. Relevant laboratory of the left nasal mass and the pathologi- data included a hemoglobin of 13.1 g/dl, cal diagnosis was consistent with angio- hematocrit of 41%, white blood cell count lymphoid hyperplasia with eosinophilia of 8,700 cells/mm3, with a differential of but could not completely rule out lym- 64% neutrophils, 30% lymphocytes, 5% phoma or granuloma. He was transferred eosinophils, and 1% monocytes. The to Ramathibodi Hospital. Physical exami- antineutrophilic cytoplasmic antibody nation revealed marked swelling, defor- (ANCA) was negative. Anti-human im- mity, and enlargement of the left side of munodeficiency virus (HIV) was the nose, obstructing the left nasal canal. negative. Computer tomography (CT) of The cervical lymph node were not en- the paranasal sinuses showed an infiltra- larged. Relevant laboratory data included tive soft tissue mass involving mainly the a hemoglobin of 14.9 g/dl, hematocrit of right nasal ala with extension to the over- 45.6%, white blood cell count of 7,710 cells/ lying skin and subcutaneous tissue. The mm3, with a differential of 65% neutro- mass occupied the anterior aspect of the phils, 25% lymphocytes, 6% monocytes, right nasal cavity and the right ethmoid 3% eosinophils, and 1% basophils. ANCA sinus causing obstructive sinusitis of the and anti-HIV were negative. A CT of the right maxillary sinus. Repeat incisional paranasal sinuses revealed a homogenous biopsies were taken from the anterior end enhancing, infiltrative soft tissue mass, of the right inferior turbinate. The patho- involving the left alar nasi. A repeat biopsy logical diagnosis was rhinofacial phycomy- was performed and histopathological find- cosis. Histopathology revealed short broad ings revealed short, broad fragmented hyphae ensheathing with amorphous eosi- hyphal elements surrounded by a nophilic Splendore-Hoeppli material. Tis- Splendore-Hoeppli reaction, compatible sue eosinophilia with granulation tissue with entomophthoramycosis. Amphoteri- Vol 41 No. 4 July 2010 929 SOUTHEAST ASIAN J TROP MED PUBLIC HEALTH Fig 1–Case 3 when first seen, with swelling of Fig 2–Section of the nasal mucosa showing hy- the nasal and paranasal areas. phal elements surrounded by Splendore- Hoeppli material and inflamed stroma consisting of tissue eosinophilia (H & E, x 400). cin B at a dosage of 1 mg/kg of body weight was given over a ten-week period with good response and there was no recur- rence. Case 3 A 55-year-old Thai male came to Chantaburi Hospital in January 2000 with bilateral nalsal masses for 3 months dura- tion. The patient had no history of signifi- cant past illness, and no family history of diabetes mellitus, tuberculosis or malig- nancies. The first biopsy revealed a mid- line granuloma. His primary physician referred him to Ramathibodi Hospital. Physical examination showed swelling of the nasal and paranasal areas with redness Fig 3–The GMS stained section of nasal mu- and mild tenderness involving the cosa showing short, broad, fragmented frontoglabella (Fig 1). There were no skin hyphal elements (x 400). or mucosal ulcerations, cervical lymphad- enopathy or other clinical abnormalities. Relevant laboratory data included a hemo- sinuses revealed mucoperiosteal thicken- globin of 13.1 g/dl, hematocrit of 38.7%, ing in the right maxillary sinus and mu- white blood cell count of 10,000 cells/mm3, cosal thickening of the left maxillary, right with a differential of 66% neutrophils, 30% frontal and right ethmoid sinuses. A repeat lymphocytes, 2% eosinophils, and 2% biopsy was performed and revealed ir- monocytes. ANCA and anti-HIV were regular sparse nonseptate branching fun- negative. Radiography of the paranasal gal hyphae, which was easily seen on a 930 Vol 41 No. 4 July 2010 RHINOFACIAL ENTOMOPHTHORAMYCOSIS Gomeri Methenamine Silver (GMS) stain Splendore-Hoeppli material (Fig 2). A at low magnification. The hyphal frag- GMS-stain showed short, broad, frag- ments were ensheathed by amorphous mented hyphal elements (Fig 3). The diag- eosinophilic Splendore-Hoeppli material. nosis was rhinofacial entomophtho- The pathological diagnosis was rhinofacial ramycosis. Medical therapy included phycomycosis. Bacteriologic and myco- itraconazole for 3 months. He had a good logical cultures of the biopsy specimen response to treatment and there was no re- were performed but there was no organ- currence. ism growth. The medical treatments in- Case 5 cluded a combination of SSKI and A 30-year-old Thai male came to trimethoprim/sulfamethoxazole with a Nakhon Pathom Hospital in April 2008 gradual response to treatment by two with bilateral nasal swelling for 8 months. weeks of medication. The treatment was The patient had no history of significant continued for 12 weeks. At eight months illness in the past. There was no family follow-up there was complete recovery history of diabetes mellitus, tuberculosis without recurrence. or malignancy. The first biopsy revealed Case 4 squamous papilloma. His primary physi- A 65-year-old Thai male came to cian referred him to Ramathibodi Hospi- Samut Prakhan Hospital in January 2002 tal. Physical examination showed swelling with a complaint of a firm swelling of the and redness of the left inferior nasal turbi-