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CASE REPORT  
Year : 2020  |  Volume : 63  |  Issue : 4  |  Page : 648-650
Entomophthoromycosis in a child: Delayed diagnosis and extensive involvement


Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India

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Date of Submission07-Jan-2019
Date of Decision16-Sep-2019
Date of Acceptance30-Sep-2019
Date of Web Publication28-Oct-2020
 

   Abstract 


Entomophthoromycosis is a rare fungal infection of the skin and subcutaneous tissue occurring predominantly in tropical and subtropical regions. In children, it mostly affects the lower half of the body. With this, we report a case of Entomophthoromycosis in a 6-year-old girl who presented late with extensive involvement of the upper half of the body. She responded well to treatment with potassium iodide and itraconazole. We also reviewed cases of Entomophthoromycosis reported in children.

Keywords: Child, entomophthoromycosis, itraconazole, potassium iodide

How to cite this article:
Takia L, Jat KR, Singh A, Priya MP, Seth R, Meena JP, Bagri NK, Kabra SK. Entomophthoromycosis in a child: Delayed diagnosis and extensive involvement. Indian J Pathol Microbiol 2020;63:648-50

How to cite this URL:
Takia L, Jat KR, Singh A, Priya MP, Seth R, Meena JP, Bagri NK, Kabra SK. Entomophthoromycosis in a child: Delayed diagnosis and extensive involvement. Indian J Pathol Microbiol [serial online] 2020 [cited 2020 Nov 24];63:648-50. Available from: https://www.ijpmonline.org/text.asp?2020/63/4/648/299299





   Introduction Top


Entomophthoromycosis is a rare fungal infection of skin and subcutaneous tissue that mostly affects immunocompetent hosts; predominantly in tropical and subtropical regions.[1] It often presents with gradually progressive soft tissue swelling mimicking cutaneous malignancy and tuberculosis and correct diagnosis is often missed for a long time. It mostly affects the lower half of the body in children.

Delayed diagnosis and extensive involvement of subcutaneous Entomophthoromycosis in a 6-year-old girl prompted us to report the case. We also reviewed cases of Entomophthoromycosis reported in children.


   Case Report Top


A six years old girl from Bihar, India, was admitted with progressively increasing swelling over the right shoulder and face for the last six months [Figure 1]a. She had multiple small papular swellings over the right shoulder at the beginning, which gradually increased in size and became confluent to involve neck, face, and scalp over the next four months. It was associated with mild pain and redness of skin but not associated with fever. She visited multiple physicians and received various drugs before presenting to us but swelling persisted.
Figure 1: (a) At presentation showing diffuse swelling of face scalp and right shoulder; (b) After 30 days of therapy showing some improvement in swelling; (c) After 180 days of treatment showing marked improvement in swelling

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She had moderate malnutrition. Local examination showed a subcutaneous swelling involving skin of right shoulder, neck, face, and scalp which was indurated, non-tender, non-mobile, and was not warm to touch [Figure 1]a. The swelling involved eyelids extensively; the child had to lift the swellings with a hand for vision. Bilateral cervical lymphadenopathy was present. The rest of the general and systemic examination was normal.

Based on the above history and physical examination possibility of subcutaneous fungal infection and subcutaneous T cell lymphoma was considered.

Investigations revealed microcytic hypochromic anemia with elevated leucocytes with neutrophilic predominance. Liver function tests and renal function tests were normal. CT scan head, nasopharynx, and thorax revealed soft tissue subcutaneous swelling in the face, neck, right shoulder, and upper chest. It has no extension to sinuses and intra-cranially and there was no destruction of pterygoid plates [Figure 2].
Figure 2: CT of patient: (a) Soft tissue subcutaneous swelling of face and forehead; (b) Soft tissue subcutaneous swelling of right shoulder

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KOH staining of a skin biopsy revealed broad aseptate hyphae. Histopathological examination of skin biopsy showed mixed inflammatory cell response with giant cell, plasma cells, and eosinophils. The fungal hyphae were surrounded by eosinophilic material (splendore-hoeppli phenomenon) that is very much suggestive of Entomophthoromycosis [Figure 3].
Figure 3: Histopathological slides showing fungal-hyphae surrounded by eosinophilic material and fungal-hyphae inside giant cell

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Fungal culture of skin biopsy sample was negative. The serum immunoglobulin levels and T cell subset counts were within normal limits. HIV test was negative. We made a final diagnosis of subcutaneous Entomophthoromycosis.

The child was started on itraconazole (100 mg/day) and potassium iodide (KI) (40 mg/kg/day). Following one month of treatment, she showed a significant decrease in subcutaneous swelling [Figure 1]b. In follow up, the child was found to have hypothyroidism which responded to a thyroid supplement. After six months of antifungal therapy, the child showed almost complete disappearance of subcutaneous swelling [Figure 1]c.


   Discussion Top


Entomophthoromycosis (Basidiobolus and Conidiobolus) is a class of saprophytic fungi, which produces infections of the respiratory tract and subcutaneous tissues. These fungi are commonly found in soil, decaying organic matter such as seeds, fruits, and manure and infection usually occur through traumatic implantation of the fungus into subcutaneous tissue.[1] The cases have been reported from several parts of tropics and subtropics including Nigeria, Ghana, India, Sudan, Uganda, and Indonesia.[2]

Infection with fungi from the order Entomophthorales typically occurs in immunocompetent patients residing in subtropical and tropical climates, and present with swelling unlike Mucorales, which cause angio-invasive disease.[3] The case reports and case series of Entomophthoromycosis in children is shown in [Table 1].
Table 1: Case reports and case series of Entomophthoromycosis in children

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In addition to above, the largest series was reported from Uganda by Mugerwa et al.[13] in 1976 where 80 cases, 76% of patients were less than ten years of age, and 88% under 20 years of age were reported. The duration of symptoms ranged from two weeks to five years with a mean of six months. The common sites of involvement were buttocks (35%), thigh (26.3%), upper limb (10%), leg (7.5%), and truck (7.5%).[13] In literature, the most common site of entomophthoromycosis in children was below the waist and lower limbs. But, in our case it involved upper body including shoulder, neck, and scalp.

Entomophthoromycosis can mimic subcutaneous lymphoma and sarcoma, which are its important differential diagnosis. Confirmatory diagnosis is mostly achieved by reviewing histopathology of tissue that shows dense eosinophilic granular infiltrates surrounding hyphal elements, the so-called Splendore–Hoeppli phenomenon.[9] The inflammatory infiltrate is mixed with eosinophils, histiocytes, neutrophil, lymphocytes, plasma cells, and giant cells. This reaction is not seen in mucormycosis. Patients may have leukocytosis and peripheral eosinophilia. Tissue culture can identify species.

Therapeutic drug and duration varied in reported cases in children [Table 1]. Potassium iodide and itraconazole are found to be the most appropriate regimen. Guarro et al.[14] performed susceptibility testing on nine Basidiobolus spp. and eight Conidiobolus spp. isolates. The geometric mean MIC values for Basidiobolus spp. were lower than for Conidiobolus spp. itraconazole, 1.8 vs. 11.3 lg/mL; ketconazole, 1.0 vs. 20.7 lg/mL; miconazole, 3.9 vs. 11.3 lg/mL; amphotericin B, 2.7 vs. 3.1 lg/mL; fluconazole, 14.8 vs. 107.5 lg/mL; and flucytosine, 165.9 vs. 234.6 lg/mL. There is no clinical experience in treating Entomophthoromycosis with voriconazole. Krishnan et al.[15] had success with potassium iodide at 40 mg/kg/day in 9 of 10 patients of either conidiobolomycosis or basidiobolomycosis over a 4-year period.

Hence, in light of available evidence, we started our patient on itraconazole and potassium iodide, and following every month of treatment, she showed significant improvement. The index case required a long (six months) therapy as the response was gradual.


   Conclusion Top


We conclude that young patients can have a subcutaneous fungal infection which can mimic malignancies like subcutaneous lymphoma or sarcomas. A high index of suspicion is required to diagnose Entomophthoromycosis to avoid unnecessary delays in diagnosis and differentiation from TB or malignancy.

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.
Sujatha S, Sheeladevi C, Khyriem AB, Parija SC, Thappa DM. Subcutaneous zygomycosis caused by Basidiobolus ranarum-A case report. Indian J Med Microbiol 2003;21:205-6.  Back to cited text no. 1
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2.
Williams AO. Pathology of Phycomycosis due to species Entomophthora and Basidiobolus species. Arch Path 1969;87:13-20.  Back to cited text no. 2
    
3.
Kudrimoti JK, Patil SS, Khandekar SS, Khandekar MM, Puranik SC. Subcutaneous Entomophthoromycosis mimicking soft tissue tumour: Report of two cases in Maharashtra, India. Paripex Indian J Res 2014;3:301-4.  Back to cited text no. 3
    
4.
Reddy PR, Gaddi D, Paga U, Kumar V. A rare case of subcutaneous entomophthoromycosis in a 11 years old child mimicking soft tissue tumor treated with itraconazole: Case report. J Med Dent Sci 2015;4:2626-9.  Back to cited text no. 4
    
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Anand M, Deshmukh SD, Pande DP, Naik S, Ghadage DP. Subcutaneous zygomycosis due to basidiobolus ranarum: A case report from Maharastra, India. J Tropical Medicine 2010. Article ID 950390. doi: 10.1155/2010/950390.  Back to cited text no. 5
    
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Al-Qahtani SM, Alsuheel AM, Shati AA, Mirza NI, Al-Qahtani AA, Al-Hanshani AA, et al. Case reports: Gastrointestinal basidiobolomycosis in children. Curr Pediatr Res 2013;17:1-6.  Back to cited text no. 6
    
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Raveenthiran V, Mangayarkarasi V, Kousalya M, Viswanathan P, Dhanalakshmi M, Anandi V. Subcutaneous entomophthoromycosis mimicking soft-tissue sarcoma in children. J Pediatr Surg 2015;50:1150-5.  Back to cited text no. 7
    
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Gordon CL, Whiting S, Haran G, Ward A, Coleman M, Baird R, et al. Entomophthoromycosis caused by Basidiobolus ranarum in tropical northern Australia. Pathology 2012;44:375-9.  Back to cited text no. 8
    
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Das P, Vijay MK, Joshi P, Yadav R, Singh G. Histological identification of Entomophthoromycosis in biopsy samples is required. Indian J Pathol Microbiol 2014;57:514-6.  Back to cited text no. 9
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Kumaravel S, Bharath K, Rajesh NG, Singh R, Kar R. Delay and misdiagnosis of basidiobolomycosis in tropical South India: Case series and review of the literature. Paediatr Int Child Health 2016;36:52-7.  Back to cited text no. 10
    
11.
Bhalla S, Srivastava VK, Gupta RK. Rhinofacial entomophthoramycosis: A rare fungal infection in an adolescent boy. Indian J Pathol Microbiol 2015;58:402-3.  Back to cited text no. 11
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12.
Arora P, Sardana K, Bansal S, Garg VK, Rao S. Entomophthoromycosis (basidiobolomycosis) presenting with “saxophone” penis and responding to potassium iodide. Indian J Dermatol Venereol Leprol 2015;81:616-8.  Back to cited text no. 12
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13.
Mugerwa JW. Subcutaneous phycomycosis in Uganda. Br J Dermatol 1976;94:539-44.  Back to cited text no. 13
    
14.
Guarro J, Aguilar C and Pujol I. In-vitro antifungal susceptibilities of Basidiobolus and Conidiobolus spp. Strains. J Antimicrob Chemother 1999;44:557-60.  Back to cited text no. 14
    
15.
Krishnan SG, Sentamilselvi G, Kamalam A, Das A, Janaki C. Entomophthoromycosis in India-a 4-year study. Mycoses 1998;41:55-8.  Back to cited text no. 15
    

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Correspondence Address:
Kana R Jat
Department of Pediatrics, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_20_19

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