|Year : 2014 | Volume
| Issue : 1 | Page : 46-48
Mucormycosis: A rare case report
Geetakiran Arakkal, Harish Kumar Kasetty, Sudhavani Damarla, Sudharani Chintagunta
Department of Dermatology, Venereology and Leprosy, Gandhi Medical College, Hyderabad, Telangana, India
|Date of Web Publication||18-Feb-2015|
Professor of Dermatology, Venereology and Leprosy, Gandhi Medical College, Hyderabad, Telangana, India. House No. 2, Vayupuri, Sainikpuri, Hyderabad, Telangana - 500 094
Source of Support: None, Conflict of Interest: None
Mucormycosis is a potentially life-threatening and emerging angioinvasive fungal infection with a high mortality rate. Mucormycosis is caused by the ubiquitous filamentous fungi of the Mucorales, order of the class of Zygomycetes. They commonly occur in patients with uncontrolled diabetes mellitus, renal insufficiency, organ or stem cell transplant, malignancy, chronic use of iron-chelating agents and immunocompromised patients. Mucormycosis is being increasingly recognized in India as well, mainly in patients with uncontrolled diabetes or trauma. The infection is acquired by the inhalation of spores or by direct inoculation of the fungus into the damaged skin or mucosa. The common reported sites of mucormycosis are the sinuses, lungs and skin. The difficulty and delay in diagnosis due to its rarity usually results in a poor prognosis. To reduce the mortality and morbidity, early diagnosis and aggressive treatment are very important. We report a case of oral mucormycosis in a patient with diabetes mellitus involving the nasal cavity and maxillary and ethmoidal sinuses eroding the hard palate.
Keywords: Diabetes mellitus, mucormycosis, maxillary sinuses, oral cavity
|How to cite this article:|
Arakkal G, Kasetty HK, Damarla S, Chintagunta S. Mucormycosis: A rare case report. Int J Oral Health Sci 2014;4:46-8
| Introduction|| |
Mucormycosis is a highly angioinvasive and a relentlessly progressive condition resulting in high morbidity and mortality. Mucormycosis represents an acute life-threatening opportunistic fungal infection with a high mortality rate caused by a group of distinctive mycoses that are ubiquitous, saprophytic fungi belonging to the class zygomycetes, order mucorales, family Mucoraceae, predominantly occurring in diabetics. 
Although the term phycomycosis and zygomycosis are occasionally used, mucormycosis is the most frequently used term, and it was first described by Paultauf in 1885.  It is the third common fungal infection after candidiasis and aspergillosis in diabetics and post-allogenic stem cell transplant patients. Up to 50% of patients presenting with mucormycosis are diabetics. 
Common predisposing factors include diabetes mellitus, neutropenia, desferoxamine use associated with dialysis, malnutrition, malignancy, renal failure and immunosuppressive therapy. 
Based on their anatomical location, six different clinical forms: Rhinocerebral, pulmonary, cutaneous, gastrointestinal, disseminated and miscellaneous, Involvement of bone or kidney, have been described. 
It is usually found in the soil, and can also be cultured from the nasal mucosa of healthy individuals in whom no signs of infection are seen.  The infection is acquired by the inhalation of spores or by direct inoculation of the fungus into the damaged skin or mucosa.  Even though the organisms are aerobic, they can survive up to 5 days in vitro. 
The most commonly isolated genera are Rhizopus, Rhizomucor and Absidia. ,, Mucormycosis involving the facial, palatal, orbital, paranasal sinus or cerebral regions is termed as rhinocerebral mucormycosis. 
The fungus causing the infection invades the arteries, forms thrombi within the blood vessels that reduce blood supply and causes necrosis of the hard and soft tissues. Infected tissue may appear to be normal initially and later progress to an erythematous phase with or without edema, before turning violaceous and finally forming a black necrotic eschar. Tissue necrosis is the clinical hallmark of invasive mucormycosis due to angioinvasion and subsequent thrombosis.
A high index of suspicion is required for an early diagnosis and initiation of adequate therapy for a good prognostic outcome. 
| Case Report|| |
A 65-year-old male farmer presented with the chief complaint of painful ulcerations over the hard palate of 6 months duration, which was gradually increasing in size. He also complained of nasal stuffiness and blackish discharge from the ulcer along with regurgitation from the nose on taking liquids, pain and loosening of the right upper molar. There was no history of epistaxis or loss of weight. He was a known diabetic with poor control for the past 6 years. On general examination, there was no pallor, icterus, cyanosis, clubbing or lymphadenopathy.
Oral examination revealed a well-circumscribed, painful, punched-out ulcer over the anterior aspect of the hard palate of size 3 cm × 3 cm × 0.25 cm in depth with yellowish black discoloration of the base. Another well-circumscribed ulcer of size 2 cm × 2 cm was seen near the right molar covered with yellowish black slough. The ulcers did not bleed on touch and the gums were normal [Figure 1] and [Figure 2]. Nasal examination showed normal external framework. Both nostrils were filled with thick yellowish black secretions. There was perforation of the nasal septum. The lateral wall of the nose and floor of the nose were normal.
|Figure 1: Two ulcers over the hard palate exposing the maxillary alveolar ridge covered with necrotic slough|
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|Figure 2: Two punched-out ulcers over the anterior aspect of the hard palate and right molar covered with yellowish black slough|
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Routine investigations were normal. Fasting blood sugar was 128 mg% and post-lunch blood sugar was 281 mg%. Venereal disease reseach laboratory (VDRL) and HIV 1 and 2 were non-reactive and hepatitis B surface antigen was negative. X-ray chest, electrocardiogram and ultrasound abdomen were normal.
A computed tomography (CT) scan showed moderate mucosal thickening with air foci in the right maxillary sinus obliterating the right maxillary antrum, moderate mucosal thickening of the ethmoidal and sphenoidal sinus and minimal mucosal thickening of the left maxillary and frontal sinus. There was mild hypertrophy of the inferior turbinate along with right sclerosing mastoiditis and chronic suppurative otitis media.
Histopathology of the ulcer from the hard palate showed hyperplastic squamous epithelium with subepithelial fibrosis and chronic inflammatory cells [Figure 3]. There was degenerating food particles and numerous non-septate branching fungal hyphae suggestive of mucormycosis.
|Figure 3: Typical characteristic broad, thin-walled, pleomorphic fungal hyphae with irregular contours (hematoxylin and eosin ×40)|
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He was started on amphotericin B (0.8 mg/kg/day) along with insulin. Presently, his sugar levels are under control and he is showing good response to therapy.
| Discussion|| |
Mucormycosis is an angioinvasive fungal infection seen in developing countries following uncontrolled diabetes. Diabetic patients have low immunity to resist mucormycosis due to the inability to inhibit Rhizopus in vitro and also reduced phagocytic ability of the granulocytes. ,
The other predisposing conditions include malignant hematological diseases with or without stem cell transplantation, neutropenia, prolonged use of corticosteroids, intravenous drug abuse, neonatal prematurity, iron overload and malnourishment. 
In most of the immunologically competent hosts, these spores are contained by a phagocytic response. Germination continues if the phagocytic response fails, leading to the development of hyphae; thereby, infection becomes established. The hyphae invade the arteries and propagate within the vessels, causing thrombosis, ischemia and infarction of the affected tissues.
A CT scan shows opacification of the paranasal sinuses and thickening of the mucosa and bone destruction. However, a definitive diagnosis of mucormycosis can only be made by a biopsy that identifies the characteristic hyphae and by culture.  Histologically, mucormycosis is characterized by extensive tissue necrosis and the presence of numerous large non-septate hyphae, with budding and dichotomous branching, giving a ribbon-like appearance. Therefore, the diagnosis of the disease is mainly based on history, clinical appearance, biopsy and imaging studies.
Successful management of mucormycosis consists of treating the underlying disease and aggressive surgical debridement along with systemic antifungal therapy. The use of amphotericin B in patients with mucormycosis has a survival rate of up to 72%, which is a widely published and accepted treatment.  More recently, hyperbaric oxygen therapy has also been used. Treatment of mucormycosis must be initiated at the earliest as it progress relentlessly and has a fatal outcome.
We hereby stress the importance of early diagnosis of mucormycosis to limit the dissemination of infection, thereby preventing morbidity and mortality. Rapid diagnosis, correcting the underlying disorder and aggressive management are necessary for a favorable outcome.
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[Figure 1], [Figure 2], [Figure 3]