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 ~  Abstract
 ~ Introduction
 ~ Case Report
 ~ Discussion
 ~  References
 ~  Article Figures

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  Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 31  |  Issue : 2  |  Page : 182-184
 

Subcutaneous mucor zygomycosis with potential life-threatening visceral complication


1 Department of Surgery, Indira Gandhi Medical College and Research Institute, Pondicherry, India
2 Department of General Medicine, Mahatma Gandhi Medical College, Pondicherry, India
3 Department of Radiology, Indira Gandhi Government General Hospital, Pondicherry, India
4 Department of Pathology, JIPMER, Pondicherry, India

Date of Submission15-Aug-2012
Date of Acceptance22-Apr-2013
Date of Web Publication19-Jul-2013

Correspondence Address:
Angeline
Department of Surgery, Indira Gandhi Medical College and Research Institute, Pondicherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0255-0857.115226

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 ~ Abstract 

A mass in right supraclavicular fossa in a diabetic patient mimicking tuberculosis (TB) adenitis that ultimately proved to be subcutaneous zygomycosis. A high degree of clinical suspicion is needed for diagnosis especially when these lesions occur at typical sites for the more common indolent infections like TB. This case is being presented not only because of its rarity, but to emphasize the role of early diagnosis and appropriate treatment to prevent serious complications due to proximity to major structures. Fluconazole was used despite not being the ideal drug, solely due to cost constraints. Our patient responded well. However, we do emphasize that response to fluconazole is the exception rather than the rule.


Keywords: Fluconazole, lymph-adenitis, stridor, subcutaneous zygomycosis


How to cite this article:
Angeline, Hanifah M, Balachandran G, Rajesh N G. Subcutaneous mucor zygomycosis with potential life-threatening visceral complication. Indian J Med Microbiol 2013;31:182-4

How to cite this URL:
Angeline, Hanifah M, Balachandran G, Rajesh N G. Subcutaneous mucor zygomycosis with potential life-threatening visceral complication. Indian J Med Microbiol [serial online] 2013 [cited 2019 Sep 19];31:182-4. Available from: http://www.ijmm.org/text.asp?2013/31/2/182/115226



 ~ Introduction Top


Zygomycosis is on the rise in India. The subcutaneous form is less fatal than the rhinocerebral, pulmonary or disseminated forms, but can be fatal if in proximity to major viscera such as the trachea, great vessels in the neck, and mediastinum.


 ~ Case Report Top


A 45-year-old male agricultural worker had presented with a swelling in the right supraclavicular fossa for 6 months [Figure 1]. He had been started on Anti-Tuberculosis treatment (ATT) after biopsy confirmation of tuberculosis (TB) of lymph nodes at another facility. Patient discontinued ATT after 2 months as there was no improvement and presented to us. There was no history of trauma. He was moderately nourished. A nodular mass 10 cm × 15 cm with no skin changes was found in the right supraclavicular fossa [Figure 1]. A working diagnosis of supraclavicular lymphadenopthy due to TB, lymphoma or secondaries was made. He was diagnosed to have diabetes mellitus in this admission with fasting blood sugar of 178 mg and post prandial of 240 mg% and responded well to oral hypoglycemics. X-ray chest, erythrocyte sedimentation rate was normal. Mantoux was negative. Serology for human immunodeficiency virus was negative. Upper gastrointestinal endoscopy was normal. Fine needle aspiration cytology revealed granulomas, but no caseation or acid fast bacilli. Hence, streptomycin was given along with rifampicin, ethambutol, pyrizinamide, and isoniazid, for about a week. Meanwhile on careful clinical examination, areas of induration extending beyond the confines of the mass and a very subtle stridor precipitated on exercise was present. Direct laryngoscopy revealed normal laryngeal mucosa. These findings led us to suspect soft- tissue fungal infection. Magnetic resonance imaging revealed an ill-defined soft-tissue mass in the R supraclavicular fossa with diffuse infiltration into the tissue spaces, around the larynx, trachea and great vessels at the neck and superior mediastinum [Figure 2]. A deep biopsy revealed eosinophilic granuloma and fungal hyphae typical of zygomycosis were demonstrated by H and E, and confirmed by silver staining [Figure 3]a and b. ATT was stopped and oral fluconazole 150 mg tid (only affordable antifungal, due to cost constraints) started even before the result for fungal culture was available. However repeated tissue culture was negative for fungal growth. Acid fast bacilli could not be demonstrated in biopsy nor could be cultured. The swelling decreased within 2 weeks. Surgical debridement was deferred as clinical response was good. Moreover, surgical clearance was not possible due to proximity to vital structures in the neck. Fluconazole was continued for 6 months with complete resolution of the swelling [Figure 4] and there was no stridor on exercise. His glycemic control was acceptable with a HbA1c of 5 mg. He had no episode of diabetic keto acidosis at any time. He is on strict follow-up and there is no evidence of recurrence 12 months later.
Figure 1: Right supraclavicular swelling

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Figure 2: Magnetic resonance imaging. Soft tissue mass in the right supraclavicular area extending on to the mediastinum and around the trachea

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Figure 3: (a) Sections show extensive necrosis with occasional fragments of broad aseptate, hyphae (arrows) with Splendore-Hoeppli phenomenon H and E, ×40 (b) Sections show extensive necrosis with occasional fragments of broad, aseptate hyphae (arrows) with Splendore-Hoeppli phenomenon and granulomatous reaction. H and E, ×100 (c) (Inset) – Sections show broad, aspetate hyphae positive for silver stains. Gomori Methenamine Silver stain, ×100

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Figure 4: Complete healing with fluconazole

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 ~ Discussion Top


Zygomycosis is caused by organisms of the class Zygomycetes, often the order Mucorales and usually Rhizopus species. [1] There is a steady rise in incidence in India over the last two decades. [2] Unlike other filamentous fungi that are largely opportunistic in patients with immunodeficiency, zygomycosis can be frequently lethal in hosts with greater immunocompetency, such as those with diabetes mellitus, which has remained the biggest host association in India [2] and world wide. [3] In fact, mucormycosis was a diabetes-defining illness in our patient. The clinically accepted types are rhino cerebral, pulmonary, gastrointestinal, cutaneous, and miscellaneous. The cutaneous form can be confined to the cutaneous subcutaneous tissue, invade muscle, tendon, viscera or involve another non-contiguous site. Disease may be gradual and slowly progressive or fulminant, leading to gangrene and haematogenous dissemination. [1] The extremities are commonly involved, but the neck has been involved in only one case. [4] FNAC revealed granulomas with no caseation and negative for AFB. Deep fungal infections can closely mimic TB. Imaging was suggestive of an ill-defined hyper dense mass extending around the trachea and explaining the subtle stridor. Biopsy revealed ribbon-like, predominantly aseptate hyphae with wide-angle (45-90°) branching, the hallmark for mucorales and confirmed with silver stain. [5] Zygomycosis is comprised of two orders, Mucorales and Entomophthorales, and their distinction is possible in biopsy. In H and E-stained tissue section, the Entomophthorales demonstrate hyphal encasement by eosinophilic material. This Splendore-Hoeppli phenomenon may be the first indication that a patient has an infection with either Basidiobolus or Conidiobolus instead of one of the Mucorales, which rarely demonstrate this phenomenon in tissues. Invasion of the blood vessels (angioinvasion) by hyphal elements is generally seen in infections with the Mucorales but usually not with the Entomophthorales. Patient was started on antifungal therapy pending fungal culture report. Fungal and AFB cultures were negative on repeated occasions. The identification of the genus and species requires culture, which can be negative in 38-70%. [1] Rapidity of diagnosis, reversal of the underlying predisposing factors, appropriate surgical debridement of infected tissue, and appropriate antifungal therapy are critical for eradicating mucormycosis. If mucormycosis is suspected, initial empirical antifungal therapy should begin while the diagnosis is being confirmed, rather than waiting for the protracted series of diagnostic tests to be completed. [7] Fatality in cutaneous disease is due to invasion into adjacent vital viscera, for example, trachea, [4] major blood vessels, [3] haematogenously disseminated infection and necrotizing fasciitis. [6] All these possible fatal outcomes were anticipated and prevented in our case by early diagnosis and initiation of antifungal therapy inspite of negative culture. The antifungal fluconazole had been very effective in the 80s for the treatment of the systemic deep-seated mycoses. [7] However, recent evidence is that the azoles should not be used in treating Zygomycosis due to the lack of both in vitro and in vivo susceptibility [5] Amphotericin B and posaconazole are the only systemic antifungals effective against Mucorales now. It is important to start one of these classes of antifungal agents as soon as possible, as treatment delays are associated with increased mortality. However, amphotericin may not be widely used due to the cost [10] especially in developing nations like India [8] and China, [9] the concern for side effects in children or milder infection in relatively immunocompetent hosts where fluconazole had been used with 65% survival rate. [5] Antifungal therapy alone has been used successfully where surgical intervention was not possible or not preferable due to involvement of vital structures [5] similar to our case. Intensity of antifungal therapy often depends on the tempo of infection progression, the magnitude of clinical response in the first 7-10 days to therapy, and control of underlying disease. [10] The prognosis in cutaneous zygomycosis is better than that in the other clinical forms of the disease, but mortality still remains 10-26%. [1],[5] A recurrence of 20% in India [8] and 33% in China [9] mandates a strict follow-up for our patient. Johnson et al.[4] had reported a similar case where there was cutaneous and laryngeal infiltration, but the patient had a fatal outcome due to delay in diagnosis. We were able to avoid the inevitable fatal outcome by initiation of antifungal therapy without waiting for culture reports. Hence, a physician's early suspicion of Mucorales infection remains the most important element for early clinical diagnosis and effective preemptive treatment should be highly individualized. [10]

 
 ~ References Top

1.Skiada A, Petrikkos G. Cutaneous zygomycosis. Clin Microbiol Infect 2009;15 Suppl 5:41-5.  Back to cited text no. 1
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2.Diwakar A, Dewan RK, Chowdhary A, Randhawa HS, Khanna G, Gaur SN. Zygomycosis: A case report and overview of the disease in India. Mycoses 2007;50:247-54.  Back to cited text no. 2
[PUBMED]    
3.Roden MM, Zaoutis TE, Buchanan WL, Knudsen TA, Sarkisova TA, Schaufele RL, et al. Epidemiology and outcome of zygomycosis: A review of 929 reported cases. Clin Infect Dis 2005;41:634-53.  Back to cited text no. 3
[PUBMED]    
4.Johnson KE, Leahy K, Owens C, Blankson JN, Merz WG, Goldstein BJ. An atypical case of fatal zygomycosis: Simultaneous cutaneous and laryngeal infection in a patient with a non-neutropenic solid prostatic tumor. Ear Nose Throat J 2008;87:152-5.  Back to cited text no. 4
[PUBMED]    
5.Ribes JA, Vanover-Sams CL, Baker DJ. Zygomycetes in human disease. Clin Microbiol Rev 2000;13:236-301.  Back to cited text no. 5
[PUBMED]    
6.Spellberg B, Edwards J Jr, Ibrahim A. Novel perspectives on mucormycosis: Pathophysiology, presentation, and management. Clin Microbiol Rev 2005;18:556-69.  Back to cited text no. 6
    
7.Ikemoto H, Watanabe K, Mori T, Taniuchi A, Akahonai Y, Mikuni C, et al. Clinical study of fluconazole on deep-seated fungal infections. Jpn J Antibiot 1989;42:63-116.  Back to cited text no. 7
    
8.Vaid NS, Athavale SA, Kale OS. Mucormycosis: A retrospective study. Indian J Otolaryngol Head Neck Surg 2005;57:136-8.  Back to cited text no. 8
[PUBMED]    
9.Lu XL, Liu ZH, Shen YN, She XD, Lu GX, Zhan P, et al. Primary cutaneous zygomycosis caused by rhizomucor variabilis: A new endemic zygomycosis? A case report and review of 6 cases reported from China. Clin Infect Dis 2009;49:e39-43.  Back to cited text no. 9
[PUBMED]    
10.Kontoyiannis DP, Lewis RE. How I treat mucormycosis. Blood 2011;118:1216-24.  Back to cited text no. 10
[PUBMED]    


    Figures

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

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