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CASE REPORT
Year : 2022  |  Volume : 23  |  Issue : 1  |  Page : 64-66

Entomophthoromycosis in a young child postcleft lip surgery: A rare case with diagnostic dilemma


1 Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India
2 Department of Pediatric Medicine, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India
3 Department of Pathology, St. John's Medical College and Hospital, Bengaluru, Karnataka, India
4 Department of Microbiology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India

Date of Submission22-Apr-2021
Date of Acceptance18-Oct-2021
Date of Web Publication31-Dec-2021

Correspondence Address:
Shreya Rajashekar
Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.ijpd_44_21

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  Abstract 


Entomophthoromycosis is a rare yet potentially life-threatening, chronic deep fungal infection affecting immunocompetent host, primarily children, and young adults. The infection commonly affects trauma-prone sites mainly extremities and trunk with regional lymph node involvement. We report a 1.5-year-old child with postcleft lip surgery presenting with painless swelling on the right side of the face for the past 4 months. Although culture showed no growth in our case, histopathological examination was diagnostic of entomophthoromycosis which aided the treatment. There was partial improvement in the size of the swelling with amphotericin B and a complete resolution with oral potassium iodide.

Keywords: Amphotericin B, Entomophthoromycosis, postcleft lip surgery, potassium iodide


How to cite this article:
Rajashekar S, Srinivas SM, Shivappa SK, Tirumale R, Jagannatha B. Entomophthoromycosis in a young child postcleft lip surgery: A rare case with diagnostic dilemma. Indian J Paediatr Dermatol 2022;23:64-6

How to cite this URL:
Rajashekar S, Srinivas SM, Shivappa SK, Tirumale R, Jagannatha B. Entomophthoromycosis in a young child postcleft lip surgery: A rare case with diagnostic dilemma. Indian J Paediatr Dermatol [serial online] 2022 [cited 2022 Jan 20];23:64-6. Available from: https://www.ijpd.in/text.asp?2022/23/1/64/334675




  Introduction Top


Entomophthoromycosis (subcutaneous zygomycosis) is a deep fungal infection restricted to tropical areas of sub-Saharan Africa, Asia, and South America. It presents clinically as: subcutaneous zygomycosis, caused by Basidiobolus ranarum and rhinofacial zygomycosis caused by Conidiobolus coronatus. These occur preferentially in patients with underlying disease or defective immunity.[1] Basidiobolus is a saprobic fungus present in soil, decaying vegetable matter, excrement of reptiles, amphibians, insectivorous bat, and fish.[2] Clinically, presents with chronic subcutaneous induration commonly affecting the limbs, trunk, and rarely face. We describe a case of entomophthoromycosis postcleft lip surgery that showed good improvement with oral potassium iodide.


  Case Report Top


A 1.5-year-old healthy girl presented with progressive painless swelling over the right side of the face for the past 4 months. She had been operated for the cleft lip at 10 months of age. There was no history of prior trauma, fever and discharge from the lesion, or history suggestive of systemic involvement. Family history and personal history were noncontributory. General physical and systemic examination was normal. On cutaneous examination, well to ill defined, nontender, erythematous, woody indurated swelling measuring 10 cm × 6 cm were present on the right side of the forehead extending to the right cheek, bridge of the nose, and the upper and lower lip [Figure 1]a and [Figure 1]b. The overlying skin was not pinchable. Differential diagnoses considered were rhinocerebral mucormycosis, rhino conidiobolomycosis, sarcoidosis, chronic abscess, panniculitis, and soft tissue tumors. The ophthalmological and otorhinolaryngological evaluation showed no local invasion.
Figure 1: (a) Well to ill-defined erythematous, indurated swelling on the right side of the face, (b) Lateral view of the face showing indurated swelling

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Laboratory investigations showed microcytic hypochromic anemia, eosinophilia, and increased serum IgE levels. X-ray of the face and contrast-enhanced computed tomography of the brain and paranasal sinuses were normal. Serology was nonreactive for both HIV 1 and 2.

Tissue examination with 20% potassium hydroxide showed aseptate fungal elements [Figure 2]. Skin biopsy showed widened and thickened septae of subcutis with dense granulomatous inflammatory infiltrate extending into lobules of adipocytes along with pauciseptate hyphae [Figure 3]a and [Figure 3]b. The culture was done on two consecutive times which showed no growth. The above findings were consistent with subcutaneous zygomycosis. The child was initially started on intravenous amphotericin B for 2 weeks which showed little improvement. Later, she was started on saturated oral potassium iodide at a dose of 30 mg/kg/day with dose increment of 50 mg/week until a maximal dose of 500 mg/day was reached, this dose was maintained for 4 months and was gradually tapered and stopped. There was reduction in size and induration by more than 60% within 4 months of treatment and near resolution by 6 months [Figure 4].
Figure 2: Potassium hydroxide mount showing aseptate fungal elements

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Figure 3: (a) Widened, thickened septae of subcutis showing dense granulomatous inflammatory infiltrate extending to lobules of adipocytes (H and E, ×400), (b) Periodic acid-Schiff stain showing pauciseptate hyphae (H and E, ×400)

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Figure 4: Post 4 months of treatment showing good improvement

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  Discussion Top


In 1886, Eidam first described and isolated filamentous fungus B. ranarum from frog excrement. Phylum Zygomycota includes Entomophthorales and Mucorales.[3] Kwon-Chung, in 2012 defined a new classification of Zygomycota as basidiobolomycosis, conidiobolomycosis, and rhino conidiobolomycosis.[4] Disease is commonly acquired through trauma, scratch, postsurgery, and insect bite. There are reports of fungus being directly inoculated in the perineum after using decaying leaves for cleaning after bowel movements.[5] Thighs, buttocks, and extremities are the common sites of predilection. In our case, infection was probably acquired through cleft lip surgery. Anaparthy and Deepika, has reported a youngest female child aged 6 months with a history of painless swelling on the left knee postinsect bite diagnosed as basidiobolomycosis.[2] Males are frequently affected than females.[6] In a study by Krishnan et al., out of 10 cases of entomophthoromycosis in children the most commonly affected age group was below 10 years.[7] The disease is more commonly seen in children and less in adolescents and adults.[8]

Clinically manifests as a hardened nodule, which progresses and spreads locally. Rarely ulceration is seen. Dissemination usually does not occur.[2] Basidiobolomycosis is a common cause of subcutaneous zygomycosis in immunocompetent children, unlike rhino conidiobolomycosis, which is more frequent in adults.[9] The gold standard method for diagnosis is culture. Histopathology plays an equally important role in identification of this disease and the characteristic features include granulomatous inflammation with eosinophilia and Splendore-Hoeppli phenomenon surrounding broad branching, pleomorphic, and sparsely septate hyphae. Special stains such as periodic acid-Schiff stain show pauciseptate hyphae. Serology and DNA sequencing from tissue can be done in culture-negative which was not feasible in our case due to logistic reasons. The isolation of fungi on culture is difficult as the positivity rate is only 15%.[10]

Mendiratta et al., reported poor response with single drug itraconazole when compared to the combination of itraconazole and potassium iodide which showed significant improvement.[9] In our case, amphotericin B showed partial improvement when started initially, though it is less commonly used, it is found to be active against 50% tested isolates. Prognosis is good if subcutaneous involvement alone is present without involving other deeper structures. Although fungal culture was negative in our case, aseptate fungal hyphae on potassium hydroxide examination and classical histopathology findings confirmed the diagnosis of subcutaneous zygomycosis. Early diagnosis and accurate management can prevent devastating outcome and mortality.

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.
Chandler FW, Watts JC. Fungal diseases. In: Damjanov I, Linder J, editors. Anderson's Pathology. 10th ed. USA: St. Louis: Mosby; 1996. p. 951-62.  Back to cited text no. 1
    
2.
Anaparthy UR, Deepika G. A case of subcutaneous zygomycosis. Indian Dermatol Online J 2014;5:51-4.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Singh R, Xess I, Ramavat AS, Arora R. Basidiobolomycosis: A rare case report. Indian J Med Microbiol 2008;26:265-7.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Kwon-Chung KJ. Taxonomy of fungi causing mucormycosis and entomophthoramycosis (zygomycosis) and nomenclature of the disease: Molecular mycologic perspectives. Clin Infect Dis 2012;54 Suppl 1:S8-15.  Back to cited text no. 4
    
5.
Mugerwa JW. Subcutaneous phycomycosis in Uganda. Br J Dermatol 1976;94:539-44.  Back to cited text no. 5
    
6.
Gugnani HC. A review of zygomycosis due to Basidiobolus ranarum. Eur J Epidemiol 1999;15:923-9.  Back to cited text no. 6
    
7.
Krishnan SG, Sentamilselvi G, Kamalam A, Das KA, Janaki C. Entomophthoromycosis in India – A 4-year study. Mycoses 1998;41:55-8.  Back to cited text no. 7
    
8.
Rodrigues O, Commey JO. Basidiobolomycosis in Ghanaian children. Trop Doct 1994;24:170-1.  Back to cited text no. 8
    
9.
Mendiratta V, Karmakar S, Jain A, Jabeen M. Severe cutaneous zygomycosis due to Basidiobolus ranarum in a young infant. Pediatr Dermatol 2012;29:121-3.  Back to cited text no. 9
    
10.
Moretz ML, Grist WJ, Sewell CW. Zygomycosis presenting as nasal polyps in a healthy child. Arch Otolaryngol Head Neck Surg 1987;113:550-2.  Back to cited text no. 10
    


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



 

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