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  Table of Contents    
CASE REPORT  
Year : 2019  |  Volume : 62  |  Issue : 4  |  Page : 618-620
Invasive, gangrenous mucormycosis of arm: A fatal opportunistic infection in a highly immunocompromised host


1 Department of Orthopaedics, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India
2 Department of Pathology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India

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Date of Web Publication14-Oct-2019
 

   Abstract 


Opportunistic fungal infections occur predominantly in immunocompromised (IC) patients. Mucormycosis has the highest mortality among fungal infections. The organism is ubiquitous in its presence. The infection is commonly acquired by inhalation of fungal spores or by inoculation by direct trauma. Rhinocerebral and pulmonary mucormycosis present commonly with high mortality rates. Cutaneous mucormycosis (CM) is rarely reported and usually presents in two forms, superficial and disseminated. The superficial infection occurs commonly in immunocompetent hosts, and it can sometimes turn angioinvasive and become a fatal and very aggressive disseminated disease, especially in IC hosts. Coexistant risk factors increase the mortality rate. We report a case of posttraumatic, highly lethal, angioinvasive, and gangrenous type of CM of the arm in an IC patient. Diabetic ketoacidosis and cirrhosis due to chronic alcoholic liver disease were the risk factors.

Keywords: Angioinvasive, fungus, gangrene, mucormycosis, trauma

How to cite this article:
Kanagaraju V, Narayanasamy VK, Sukumaran S, Moorthy U, Sundar V S, Lakshmi S V. Invasive, gangrenous mucormycosis of arm: A fatal opportunistic infection in a highly immunocompromised host. Indian J Pathol Microbiol 2019;62:618-20

How to cite this URL:
Kanagaraju V, Narayanasamy VK, Sukumaran S, Moorthy U, Sundar V S, Lakshmi S V. Invasive, gangrenous mucormycosis of arm: A fatal opportunistic infection in a highly immunocompromised host. Indian J Pathol Microbiol [serial online] 2019 [cited 2019 Dec 8];62:618-20. Available from: http://www.ijpmonline.org/text.asp?2019/62/4/618/269090





   Introduction Top


Cutaneous mucormycosis (CM) is a rare and a superficial form of noninvasive opportunistic fungal infection. Rarely does it turn aggressive, leading to an angioinvasive, gangrenous mucormycosis (AIGM) with systemic dissemination in extremely immunocompromised (IC) states. The diagnosis is often delayed because of its rarity and aggressive course.[1] We report a case of posttraumatic AIGM of the arm, in an IC patient with diabetic ketoacidosis (DK) and chronic alcoholic liver disease with cirrhosis (CALDC) as dual risk factors.


   Case Report Top


A 43-year-old man with a history of fall from a two-wheeler had sustained a closed fracture of left proximal humerus and was managed conservatively in a peripheral hospital with plaster of Paris (POP) splint. He had developed fever on the 10th day, and when the POP was removed, a blister was noticed on the back of his arm. The blister ruptured and the lesion became erythematous and indurated later. On the 15th day, he became breathless and was referred to our hospital.

On arrival, he was dyspneic and febrile (100.4°F). A black eschar (15 × 15 cm) over the posterior aspect of his left arm with a central necrotic patch (7 × 7 cm) and erythematous, indurated surrounding was noted [Figure 1]a. He was on DK and suffering from CALDC. His baseline blood investigations were done (blood sugar - 356 mg/dl, urine ketones 2+, serum creatinine - 2.1 mg/dl, total bilirubin - 7.0 mg/dl, total count - 22,000/mm 3, neutrophils - 91%, and erythrocyte sedimentation rate - 105 mm/h).
Figure 1: (a) Black eschar of 15 × 15 cm with a central necrotic area of 7 × 7 cm and surrounding erythematous and indurated area in the posterior aspect of the left arm. (b) Immediately after debridement, with bleeding tissues. (c) Two days postoperative with underlying gangrenous muscles. (d) One day after the second debridement, showing extensive gangrene and cotton woolly-appearing fungal growth

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As he was looking toxic with septic features, an emergency debridement was done [Figure 1]b, and broad spectrum antibiotics were started, considering it as necrotizing bacterial cellulitis (NBC). Bacterial and fungal stains were negative for organisms. On the second postoperative day, the underlying muscles were found to be necrotic [Figure 1]c, and a redebridement was done. On the following day, the lesion had progressed to involve the entire arm with cotton woolly growth [Figure 1]d. AIGM was confirmed with repeat fungal stain [Figure 2]a and histopathological examination (HPE) [Figure 2]b, [Figure 2]c, [Figure 2]d. Infectious disease (ID) consultation was obtained and intravenous Amphotericin B was started. The lesion continued to expand despite treatment and his condition deteriorated. Fungal culture continued to be negative. Despite emergency shoulder disarticulation the lesion continued spreading involving his neck, chest, and abdominal wall. His condition continued to deteriorate with headache, disorientation, worsening respiratory distress and expired in 3 days.
Figure 2: (a) KOH mount showing broad, ribbon like hyaline, pauciseptate hyphae, with a branching pattern of 45° to 90°. (b) Broad pauciseptate hyphae within necrotic muscles (H and E, ×40). (c) Broad pauciseptate hyphae (Gomori's Methenamine Silver × 10). (d) Broad pauciseptate hyphae (Periodic acid schiff staining × 40 times magnified)

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   Discussion Top


Mucormycosis (Zygomycosis) is a life-threatening opportunistic fungal infection caused by the fungi of the order Mucorales and class Zygomycetes. They are ubiquitous and the spores are isolated from soil, decaying organic matter, excreta, and even air.[2] Mucormycosis usually presents as a rhinocerebral, pulmonary, gastrointestinal, or a disseminated disease. CM represents less than 10% of the reported cases. The disease is usually acquired either by direct inoculation (CM) or inhalation (rhinocerebral/pulmonary mucormycosis) of spores.[3] Sporadic cases of CM following an injection, elastic adhesive tape, burns, intravenous catheters, transdermal patches, and contaminated wound dressing have been reported.[3]

IC status is the most important risk factor with DK, hematological malignancies, organ transplantation, iron overload, steroid abuse, hepatic and renal failure (RF), and AIDS comprising the majority of cases. The impaired neutrophil chemotaxis and phagocytosis and the decreased pH leading to an increase in serum unbound iron in DK promote the uninhibited fungal growth.[4] The altered coagulation profile, thrombocytopenia, and deranged metabolic functions in CALDC further increase the chances of infection.[5] The survival rate of invasive mucormycosis in DM is 60%–90% and in CALDC is 11.7%. Only 2 out of 17 reported cases of invasive mucormycosis in CALDC survived.[6] The prognosis depends on the number of risk factors, two risk factors increasing the mortality rate by 3.7 times compared with one or no risk factor.[3] Our patient had both DK and CALDC as risk factors.

Primary CM usually presents in two forms. The benign, superficial CM (SCM) occurring in an immunocompetent host and the aggressive AIGM in an IC host.[1] The typical clinical presentation of CM is the necrotic eschar [Figure 1], however, this may be absent in the early stages of the disease. The presentation may range from a superficial skin involvement such as plaque, swelling, pustule, cellulitis, blister, nodule, ulceration, and ecthyma gangrenosum like lesion to deeper lesions such as necrotizing fasciitis and osteomyelitis. The arms and legs are the most common sites, but any area of the skin can be affected.[1],[3]

Deep infection resembles NBC, gangrene or a noninfectious ulcer. Hence, a high degree of suspicion is necessary, as it can turn aggressive and a delay in diagnosis may be disastrous.[1],[7] The early diagnosis can be made by direct microscopy and HPE. The fungi are visible as a pauciseptate, broad ribbon-like branching hyphae (45°–90°). In AIGM, the hyphae invade the blood vessels leading to vasculitis, hemorrhage, thrombosis, infarction, and necrosis as seen in our case [Figure 2]. Although the fungi grow in Sabouraud's dextrose agar only 30% are culture positive because the hyphae become disintegrated and nonviable during the biopsy procedure or tissue grinding process in the laboratory.[7]

The prognosis depends on the extent of fungal spread with a mortality of 10% in SCM, 26% in AIGM, and 94% in disseminated disease with an overall mortality of 31%.[8] Treatment of CM is multipronged. SCM is managed by antifungal (AF) and conservative debridement. AIGM is fulminant and very aggressive with early dissemination, and often the diagnosis is delayed. Hence, a high index of suspicion and early diagnosis along with systemic AF, radical debridement and sometimes amputation along with management of underlying IC state may be life-saving. But even with treatment, the end may be disastrous.[1],[7],[9]

Conventionally, amphotericin B (0.7–1.0 mg/kg/day) is the standard AF used. But dose should be titrated to reduce the nephrotoxic side effect of the drug in RF. Recently, liposomal formulations with comparable efficacy and low renal toxicity are used. The use of posaconazole, hyperbaric oxygen, and iron chelator in sporadic cases are reported, but their efficacy is still uncertain. Poor response to AF because of resistant strains have been reported.[10] The probable cause of death in this patient could be due to disseminated disease with multiorgan involvement. IC status with two high-risk factors (DK, CALDC), delay in presentation and diagnosis, delay in involving an ID specialist, poor response to treatment contributed to the fatal outcome in this patient. Recent reports indicate a rise in the prevalence of mucormycosis, due to an increased incidence of IC population and high-velocity injuries.[10] The awareness about the disease and a high index of suspicion when encountering an atypical lesion in an IC patient along with early aggressive treatment can be lifesaving.

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.

Acknowledgment

We would like to thank Dr. Vidyalakshmi, Pathologist for providing the pathology slides.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Ryan ME, Ochs D, Ochs J. Primary cutaneous mucormycosis: Superficial and gangrenous infections. Pediatr Infect Dis 1982;1:110-4.  Back to cited text no. 1
    
2.
Alvarez E, Sutton DA, Cano J, Fothergill AW, Stchigel A, Rinaldi MG, et al. Spectrum of zygomycete species identified in clinically significant specimens in the United States. J Clin Microbiol 2009;47:1650-6.  Back to cited text no. 2
    
3.
Petrikkos G, Skiada A, Lortholary O, Roilides E, Walsh TJ, Kontoyiannis DP. Epidemiology and clinical manifestations of mucormycosis. Clin Infect Dis 2012;54 Suppl 1:S23-34.  Back to cited text no. 3
    
4.
Rammaert B, Lanternier F, Poirée S, Kania R, Lortholary O. Diabetes and mucormycosis: A complex interplay. Diabetes Metab 2012;38:193-204.  Back to cited text no. 4
    
5.
Abbas Z, Jafri W, Rasool S, Abid S, Hameed I. Mucormycosis in patients with complicated cirrhosis. Singapore Med J 2007;48:69-73.  Back to cited text no. 5
    
6.
Elsiesy H, Saad M, Shorman M, Amr S, Abaalkhail F, Hashim A, et al. Invasive mucormycosis in a patient with liver cirrhosis: Case report and review of the literature. Hepat Mon 2013;13:e10858.  Back to cited text no. 6
    
7.
Raizman NM, Parisien M, Grafe MW, Gordon RJ, Rosenwasser MP. Mucormycosis of the upper extremity in a patient with alcoholic encephalopathy. J Hand Surg Am 2007;32:384-8.  Back to cited text no. 7
    
8.
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. 8
    
9.
Klepser M. The value of amphotericin B in the treatment of invasive fungal infections. J Crit Care 2011;26:225.e1-10.  Back to cited text no. 9
    
10.
Dai Y, Walker JW, Halloush RA, Khasawneh FA. Mucormycosis in two community hospitals and the role of infectious disease consultation: A case series. Int J Gen Med 2013;6:833-8.  Back to cited text no. 10
    

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Correspondence Address:
Vijayanth Kanagaraju
Department of Orthopaedics, PSG Institute of Medical Sciences and Research, Peelamedu, Coimbatore - 641 004, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_854_15

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