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Rhinoentomophthoromycosis: rarity revisited.

Abstract

We describe a case of rhinoentomophthoromycosis, also known as rhinophycomycosis, in an Indian man hailing from the tropical northern region of the country. The patient presented with subcutaneous swelling in the midline of the forehead, dorsum of the nose, adjoining cheek area (more on the right side), philtrum, and upper lip. Biopsy taken from the right nostril area revealed nasofacial phycomycosis. The patient was treated successfully with itraconazole, 200 mg twice daily, and potasssium iodide, 5 drops in fruit juice three times daily.

Introduction

The order Entomophthorales belongs to the subphylum Zygomycotina under the phylum Zygomycota. Zygomycosis due to entomophthoraceous fungi is caused by the species of two genera: Basidiobolusand Conidiobolus. The fungus generally affects otherwise healthy individuals, and the infection is slowly progressive, chronic, and restricted to subcutaneous tissue. Rhinocerebral mucormycosis, a more common condition, is caused by mucoraceous fungi. Zygomycosis can be distinguished from infections caused by mucoraceous fungi by a lack of vascular invasion or infarction and the production of a prolific chronic inflammatory response and Splendore-Hoeppli phenomenon around the hyphae.

Conidiobolus coronatus was first described by Rixford and Gilchrist in 1896. (1) It affects the upper respiratory tract with involvement of nasal and paranasal mucosa and extension to the skin of the nose, upper lip, and frontoglabellar area, causing a chronic granulomatous inflammatory response with grotesque disfiguration of the face. (2) Basidiobolus ranarum is a chronic inflammatory disease restricted to subcutaneous tissue of the limbs, chest, back, or buttocks, primarily seen in children and males. (3-5)

Zygomycosis affects humans, domestic and wild animals, and insects. (6) It is a cosmopolitan fungus that is more often pathogenic in wet tropical and subtropical climates. (2,3) Most cases have been reported to originate from African wet forest areas, with a few from arid zones, as well. Very few cases have been reported from the Indian tropical and subtropical region. Knowledge of this disease is imperative when dealing with patients from damp, tropical climates, since it masquerades as a tumor or deep scleroderma and is completely curable with specific treatment.

Case report

A 50-year-old man from northern India (a tropical region) presented to the ENT outpatient department with complaints of nasal stuffiness and gradually progressive swelling over the nose, adjoining cheek area, upper lip, and midforehead of 1.5 years' duration. Local examination revealed a firm, well-defined, nontender, erythematous, noncompressible, and nonreducible swelling. It was fixed to the skin over the nose, forehead, adjoining cheek area, and upper lip. Internal nasal examination revealed thickening of the ala and lateral wall of the right nasal cavity, which was obliterating the vestibule. The overlying skin was smooth and erythematous (figure 1).

Noncontrast computed tomography (CT) of the paranasal sinuses revealed mucosal hypertrophy blocking the right ostiomeatal complex, thickened ala and lateral wall of the right nasal cavity, and thickened subcutaneous tissue in the malar region (figure 2). Biopsy of the lateral wall of the nasal cavity and forehead was subjected to fungal stains: periodic acid-Schiff, Gomori methenamine silver, and Van Giesen stain. These stains showed broad aseptate hyphae enclosed in giant cells. Splendore-Hoeppli phenomenon was observed on microscopic examination. Splendore-Hoeppli phenomenon is defined as radiating or annular eosinophilic deposits of host-derived materials and possibly parasite antigens that form around fungi, helminths, or bacterial colonies in tissue. The clinical diagnosis was confirmed by histopathology as rhinoentomophthoromycosis (figure 3).

[FIGURE 1 OMITTED]

Treatment was initiated with itraconazole, 200 mg twice daily, and a saturated solution of potassium iodide, 5 drops with orange juice three times daily for 1 month. (7) The swelling drasticallyimproved after 1 month, and after 3 months of treatment, there was complete resolution (figure 4).

[FIGURE 2 OMITTED]

[FIGURE 3 OMITTED]

Discussion

The diagnosis in this case was based on clinical features and histopathologic examination using fungal stains. The patient lived in a rural, tropical region and was exposed to mold in the environment such as is usually found in soil, decaying plant debris, insects, and domestic animals. The swelling in our patient started from the nose, as is generally seen. Inhalation of spores and trauma in the form of an insect bite or nose picking seems to be the primary route of infection, thus making the nose the primary site of infection and manifestation. Some reports also suggest pulmonary and pericardial involvement. (8,9)

The infection clinically appeared to be caused by Conidiobolusbecause of its peculiar sites of presentation. Although very few cases have been reported in the world literature, better mycologic diagnostic tests, knowledge of the disease, and its mention alongside mucormycosis in the ENT textbooks, could lead to more frequent diagnosis of this disease.

[FIGURE 4 OMITTED]

References

(1.) Pujary P, Pujary K, Mahesh SG, Valiathan M. Conidiobolus coronata granuloma of left inferior turbinate:A rare presentation. J Laryngol Otol 2008;122(7):e15.

(2.) Martinson FD. Chronic phycomycosis of the upper respiratory tract: Rhinophycomycosis entomophthorae. Am J Trop Med Hyg 1971;20(3):449-55.

(3.) Gugnani HC. Entomophthoromycosis due to Conidiobolus. Eur J Epidemiol 1982;8(3):391-6.

(4.) Werth R, Sabwe-Mubangu J, Gatti F, Bastin JP. Second case of rhinoentomophthoromycosis caused by Entomophthora coronata observed in Republic of Zaire [in French]. Ann Soc Belg Med Trop 1972;52(4):343-55.

(5.) Nathan MD, Keller AP Jr., Lerner CJ, Davis JC. Entomophthorales infection of the maxillofacial region. Laryngoscope 1982;92(7 Pt 1):767-9.

(6.) Mendoza L, Alfaro AA. Equine subcutaneous zygomycosis in Costa Rica. Mykosen 1985;28(11):545-9.

(7.) Restrepo A. Treatment of tropical mycoses. J Am Acad Dermatol 1994;31(3 Pt 2):S91-102.

(8.) Ribes JA, Vanover-Sams CL, Baker DJ. Zygomycetes in human disease. Clin Microbiol Rev 2000;13(2):236-301.

(9.) Walsh TJ, Renshaw G, Andrews J, et al. Invasive zygomycosis due to Conidiobolus incongruus. Clin Infect Dis 1994;19(3):423-30.

Naresh Bhatia, MS; Charu Singh, MS; Rahul Kawatra, MS; Seraj Alam, MS; Ruchir Dhawan, MS

From the Department of Otorhinolarynogology-Head and Neck Surgery, King George's Medical University (Dr. Bhatia), and the Department of Otorhinolarynogology-Head and Neck Surgery, ERAs Lucknow Medical College (Dr. Singh, Dr. Kawatra, Dr. Alam, and Dr. Dhawan), Lucknow, India.

Corresponding author: Dr. Charu Singh, F-4; Surya Apartments-II Sector-21; Indira Nagar, Lucknow, Uttar Pradesh, India. E-mail: charu_panki@rediffmail.com
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Title Annotation:ORIGINAL ARTICLE
Author:Bhatia, Naresh; Singh, Charu; Kawatra, Rahul; Alam, Seraj; Dhawan, Ruchir
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Geographic Code:9INDI
Date:Jun 1, 2010
Words:1034
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