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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 3  |  Issue : 2  |  Page : 118-121

Rhinoentomophthoromycosis in Northern Nigeria: Report of two cases


1 Department of Surgery, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria
2 Department of Pathology, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria

Date of Submission02-Oct-2015
Date of Acceptance30-May-2016
Date of Web Publication21-Jun-2016

Correspondence Address:
Dr. Iliyasu Yunusa Shuaibu
Department of Surgery, Ahmadu Bello University Teaching Hospital, Shika, Zaria
Nigeria
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DOI: 10.4103/2384-5147.184380

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  Abstract 

Rhinoentomophthoromycosis is a rare granulomatous disease restricted to the nose and its surrounding tissues. The disease occurs mostly in tropical rainforest of Africa, India, South and Central America, and South East Asia. Etiological diagnosis of the disease may be difficult because the fungal element is rarely found in the tissue and if present may be fragmented. Two cases of managed subcutaneous zygomycosis (rhinoentomophthoromycosis) from the Savannah region of Northern Nigeria are presented. A review of clinical presentation, investigations, and treatments outcome are described. High index of suspicion is required so that treatment can be started early to avoid attendant consequences and improve the outcome.

Keywords: Northern Nigeria, rhinoentomophthoromycosis, treatment outcome


How to cite this article:
Bakari A, Shuaibu IY, Usman MA, Mayun A, Ahmed SA. Rhinoentomophthoromycosis in Northern Nigeria: Report of two cases. Sub-Saharan Afr J Med 2016;3:118-21

How to cite this URL:
Bakari A, Shuaibu IY, Usman MA, Mayun A, Ahmed SA. Rhinoentomophthoromycosis in Northern Nigeria: Report of two cases. Sub-Saharan Afr J Med [serial online] 2016 [cited 2020 Jun 5];3:118-21. Available from: http://www.ssajm.org/text.asp?2016/3/2/118/184380


  Introduction Top


Rhinoentomophthoromycosis is a rare granulomatous disease mainly restricted to the nose and its surrounding tissues. [1] The disease is known as Martinson's disease, named after Frank D. Martinson who first described it at the University College Hospital Ibadan, in 1963, and it bears his name Martinson's disease. [2] The causative organism is a fungus belonging to the order entomophthorales which is also part of the zygomycetes class. Entomophthora coronata, the most common agent, can also infect reptiles, chimpanzees, dolphins, and horses producing granulomas in the air passages and surrounding tissues. [2]

The disease begins in the nose involving soft tissues, and from there, it may disseminate locally spreading to the facial muscles or paranasal sinuses. The disease may become systemic but this is usually uncommon. [3],[4],[5]

The disease occurs mostly in tropical rainforest of Africa, India, South and Central America, and South East Asia. [1],[2] We report two cases of this rare subcutaneous zygomycosis from Giwa local government of Kaduna state which is located in the Savannah region of Northern Nigeria.


  Patients and Methods Top


This case report involved two patients who presented to ear, nose, and throat unit of Ahmadu Bello University Teaching Hospital Shika in Giwa local government between November 2011 and December 2013. Informed consent was obtained from the patients. All the patients had clinical, radiologic, and histopathological confirmation of the disease. They were treated with similar drugs and subsequently followed up for minimum of 2 years.


  Case Reports Top


Case 1

A 40-year-old farmer presented on November 8, 2011, with 3 months history of progressive painless swelling of the nose and forehead. He had preceding history of recurrent nasal congestion and blockage with associated numerous episodes of spontaneous epistaxis that lasted for a month. He rears animals and usually gets in contact with them. He also farm sorghum and millet [Table 1]. On examination, he was found to have midline nontender hard swelling on the frontal region extending down the nasal bridge. The right inferior turbinate was atrophied and covered with crust. The left inferior turbinate was engorged. The hard palate was pale with numerous vascular channels. He had purulent postnasal discharge with granular posterior pharyngeal wall [Figure 1].
Figure 1: Midline hard swelling on the frontal region extending down the nasal bridge

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Table 1: Characteristic features of the cases


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An assessment of rhinoentomophthoromycosis was made with differential of Wegener's granulomatosis and rhinoscleroma. He had erythrocyte sedimentation rate (ESR) of 45 mm/h, normal full blood count (FBC) and differential, urea, electrolyte and creatinine, and random blood sugar. HIV screening was negative. Computerized tomographic scan of his paranasal sinuses revealed a right antral soft tissue mass with pansinusitis. Biopsy of this mass was taken during examination under anesthesia (EUA) procedure of the nose and nasopharynx. Histology report showed stratified squamous epithelium overlying densely fibrocollagenous stroma that is composed of numerous granuloma made up of predominantly multinucleate giant cells, fungal spores, and occasional hyphae and lymphoplasma cells admixed with polymorphs including eosinophils [Figure 2]. Fungal culture was negative.
Figure 2: Section showing Langhan giant cell containing fungal hyphae (PAS, ×400)

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The patient was treated with cotrimoxazole 800 mg/160 mg twice daily, itraconazole 200 mg twice daily, and potassium iodide 1 ml (42 mg/kg/day) three times daily. He did very well with symptoms resolving after 6 weeks. He was followed up for 24 months with no recurrence of symptoms.

Case 2

A 42-year-old male presented to ENT clinic on January 15, 2013, with 6 weeks history of progressive swelling of forehead and nose and later noticed similar swelling on the upper lip. He had associated history of rhinorrhea and nasal blockage with no history of epistaxis. He is a Fulani man that farm and rear animals (cows, sheep, goats, and chickens).

On examination, he was found to have midline nontender woody hard swelling on the frontal bone, nasal bridge, and the upper lip. Both inferior turbinates were engorged completely obliterating both nasal cavities and no obvious intranasal mass noted. He had granular posterior pharyngeal wall and copious yellowish postnasal discharge [Figure 3].
Figure 3: Midline woody hard swelling on the frontal bone, nasal bridge, and the upper lip

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An assessment of rhinoentomophthoromycosis was made with differential of rhinoscleroma and sinonasal tumors. His ESR was 75 mm/h. FBC and differential, urea/electrolyte/creatinine, and random blood sugar were normal. HIV screening was negative. Fungal culture did not yield any organism.

Plain radiograph of paranasal sinuses showed engorged inferior turbinates and opacification of both maxillary antra. The patient had EUA of the nose, nasopharynx, and nasal submucosal Biopsy was taken. Histology report showed mucosa lined by stratified squamous epithelium with multiple granulomas consisting of multinucleated giant cells and polymorphs including eosinophils. Fungal spore and occasional hyphae were also seen [Figure 4].
Figure 4: Section showing septate fungal hyphae at the center (PAS, ×400)

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The patient was placed on potassium iodide 1 ml (42 mg/kg/day) three times daily, cotrimoxazole 800 mg/160 mg and itraconazole 200 mg twice daily. He did very well within the first 4 weeks and was followed up for 24 months with no recurrence of symptoms.


  Discussion Top


Entomophthoromycosis is a chronic granulomatous fungal infection with varied presentation as subcutaneous, mucocutaneous, and visceral infections. The majority of the subcutaneous infection caused by entomophthoralean fungi involves Basidiobolus spp., Conidiobolus coronatus, or Conidiobolus incongruous. [6]

Mode of transmission of this disease may be through insect bite and transepidermal inoculation with contaminated vegetable matter, but traumatic implantation has also been suggested to play a role. [7] The predisposing factors of this diseases may include uncontrolled diabetes mellitus and prolonged steroid therapy in cases of lymphoma, leukemia, and renal transplant. [8] None of the two patients presented here has any of these factors.

It may begin as submucosal granuloma of the inferior turbinate presenting as a very firm or woody hard smooth lobulated swelling spreading through the natural ostium to the paranasal sinuses and the subcutaneous tissue of the face. It can also spread through interosseous suture and foramina to involve internally the nasopharynx, oropharynx, palate and externally the dorsum of the nose, glabella, central frontal region, cheek, eyelids, and upper lip. [1],[2] Although the disease affects underlying tissues, the bone and skin are spared. [3] In our patients, the disease involved inferior turbinates, paranasal sinuses, and palate with woody hard swelling on the nasal dorsum, forehead and upper lip which is consistent with the first description of the disease by Martinson. [2]

Patients may present with preceding history of progressive nasal obstruction, rhinorrhea, and rarely epistaxis. These may be followed by swelling of the nasal dorsum and other areas. Our patients also presented with nasal blockage, rhinorrhea, and epistaxis (first case) followed by progressive painless swelling of the dorsum of the nose, forehead, and upper lip (in one patient).

In addition to clinical diagnosis, mycological and histological confirmation is essential. [1] Etiological diagnosis of the disease may be difficult because the fungal element is rarely found in the tissue and if present may be fragmented. [8] Another reason is that fungal hyphae may be present only in some parts of the specimen. [8] Biopsy is better taken either intranasal or through the buccal sulcus. [2] This is to ensure that material taken is from the growing edges of the lesion where viable fungi are commonly found. The histology may show multiple granuloma, fibroblastic proliferation, chronic inflammatory pattern with an eosinophilic precipitate (Splendore-Hoeppli phenomenon). [1],[9] Isolation of fungi on culture is difficult as it is positive in only 15% of cases. [10] This is also the findings in our patients in which no fungi was isolated on culture in the two cases.

Radiographic evaluation of the paranasal sinuses may show the involvement of the paranasal sinuses, extension to nasopharynx and also rule out bony involvement. [2]

Treatment of rhinoentomophthoromycosis is difficult especially if the disease is diagnosed late. [1] Various treatments have been mentioned in the literature. Potassium iodide in the dose of 42 mg/kg may be suitable for use in the developing countries because of wide availability and low cost. [1],[2] In many parts of the world, cotrimoxazole, ketoconazole, itraconazole, and amphotericin B in addition to potassium iodide are the drug used in treatment of rhinoentomophteromycosis. [11] The two patients in this case were treated with combination of cotrimoxazole, itraconazole, and potassium iodide as described in the literature.

The role of surgery in the treatment of rhinoentomophteromycosis is very limited and may involve excision of the lesion in those that present early. [2]


  Conclusion Top


Rhinoentomophthoromycosis is predominantly a disease that has been ascribed to dwellers of the tropical rain forest. However, these two cases confirm that the diseases can occur among those living outside this region and therefore a high index of suspicion is required so that treatment can be started early to avoid attendant consequences and improve the outcome. To the best our knowledge, this is the first report of subcutaneous zygomycosis from the Savannah region of Northern Nigeria.

Declaration of Patient Consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial Support and Sponsorship

Nil.

Conflicts of Interest

There are no conflicts of interest.

 
  References Top

1.
Thomas MM, Bai SM, Jayaprakash C, Jose P, Ebenezer R. Rhinoentomophthoromycosis. Indian J Dermatol Venereol Leprol 2006;72:296-9.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
2.
Martinson FD. Clinical, epidemiological and therapeutic aspects of entomophthoromycosis. Ann Soc Belg Med Trop 1972;52:329-42.  Back to cited text no. 2
[PUBMED]    
3.
Ochoa LF, Duque CS, Velez A. Rhinoentomophthoromycosis. Report of two cases. J Laryngol Otol 1996;110:1154-6.  Back to cited text no. 3
    
4.
Restrepo A. Treatment of tropical mycoses. J Am Acad Dermatol 1994;31(3 Pt 2):S91-102.  Back to cited text no. 4
    
5.
Shetty D, Sheetal R, Shreepad S, Jagdish CK, Hema K, Shreevidya B. Rhinofacial zygomycosis - A rare and interesting case. Otolaryngol Online J 2004;4:4.  Back to cited text no. 5
    
6.
Agrawal S, Meshram P, Qazi MS. Rhinoentomophthoromycosis: A rare case report. Indian J Med Microbiol 2013;31:401-3.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
7.
Khan ZU, Khoursheed M, Makar R, Al-Waheeb S, Al-Bader I, Al-Muzaini A, et al. Basidiobolus ranarum as an etiologic agent of gastrointestinal zygomycosis. J Clin Microbiol 2001;39:2360-3.  Back to cited text no. 7
    
8.
Youssef NM, Shaheen MA, Zu-El Fakkar N, Ghadiry G, El- Darouty E, Massoud MA, et al. A Clinicopathological and mycological study of chronic rhinofacial zygomycosis (Rhinoentomophthoromycosis). Egypt Dermatol Online J 2005;1:4.  Back to cited text no. 8
    
9.
Ribes JA, Vanover-Sams CL, Baker DJ. Zygomycetes in human disease. Clin Microbiol Rev 2000;13:236-301.  Back to cited text no. 9
    
10.
Kaufman L, Mendoza L, Standard PG. Immunodiffusion test for serodiagnosing subcutaneous zygomycosis. J Clin Microbiol 1990;28:1887-90.  Back to cited text no. 10
    
11.
Kabra SK, Jain Y, Sudhin T, Iyer KV, Ninan SA, Seth V. Successful treatment of entomophthoromycosis with itraconazole. Indian Pediatr 1998;35:163-6.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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