Disseminated systemic mucormycosis. (A case report).
The infection caused by fungi of the class phycomycetes has continued to be infrequent but causes significant morbidity which may go undetected. The term phycomycosis (mucormycosis) refers to a group of fungal diseases caused by several members of the class phycomycetes.,  Initially localised subcutaneous granulomas may occur which have mainly been reported from South East Asia and Africa, ; due to usage of antibiotics and steroids, systemic form may be occurring oftener.
The commonest sites of initial invasion are the paranasal sinuses, gastro-intestinal tract and lungs. It spreads by direct extension but dissemination to distant sites occurs by both haematogenous and lymphogenous routes,  Any factor compromising host immunity e.g. diabetes, malignancy, use of steroids or antimitotic drugs etc. may predispose in this.,  The diagnosis depends on recognition of the fungus in specimens of tissue or of body fluids.
A 14 year old, male student coming from rural Maharashtra was seen in the E.N.T. Unit of our institute on 11th January, 1977 with recurrent ear discharge since childhood and high fever with rigors for 15 days. On clinical examination, he was fully conscious, with tenderness over the left mastoid region and foul smelling discharge from both ears. Neurological examination revealed 6th nerve weakness but there was no neck stiffness. While both plantars were extensor, other deep reflexes were sluggish. There was no other systemic abnormality. With a suspicion of cerebral abscess, a left posterior parietal burr hole was done. Cerebrospinal fluid was blood-stained and under low pressure; microscopically it showed no cells with sugar-92 mg% and protein-17 mg%. A ventriculogram done then (on 12th January, 1977) was normal. For otorrhoea, left radical mastoidectomy was done on 17th January, 1977 and 5 ml of pus was drained from an extradural abscess, which grew Pseudomonas but blood cultures were negative. The toxemia responded to gentamicin (though not to penicillin and chloramphenicol) and the patient became afebrile for next 10 days after which toxemia and breathlessness reappeared with meningeal signs on 2nd February, 1977. On examination, both plantars were again extensor and left 6th nerve weakness had persisted. A chest radiograph showed left sided pneumothorax which was tapped. Again the patient was treated with higher antibiotics without any improvement. The blood cultures and mastoid swabs taken on the same day grew pure growth of mycelial forms of mucor. At this stage, he was transferred to the chest unit and was treated with intravenous amphotericin B, 25 mg daily for 12 days and heparin 2500 units 8 hourly, with clinical and radiological improvement. However, five days after discontinuing amphotericin B, there was a recurrence of pneumothorax and neurological signs; CSF examination now on 4th March, 1977 showed a few hyphae. Amphotericin B was restarted with very good response. A localized empyema on the left side was discovered and was drained by frequent tapping and treated with a prolonged course of amphotericin B.
Over this period, the patient showed moderate anaemia (Haemoglobin: 7.8 gm%), but there was never any leucocytosis. Sedimentation rate was intermittently high. Serum proteins showed low albumin (1.7 gm%) and normal gamma globulins (1.15 gm%). Cerebrospinal fluid examination on 8th March, 1977 showed sugar 10 mg%, protein 530 mg% and a large number of cells, 90% being polymorphs and remaining lymphocytes. Ear swabs grew Pseudomonas again and pleural fluid examination revealed (in March) an exudate with many pus cells, with predominent polymorphs (70%), eosinophils (20%); the fluid grew E. coli, Klebsiella and also show-ad fungal hyphae at one stage. Serial radiographs showed the following:
Chest: 31-12-1976-Multiple nodular shadows in the left upper and middle zones, with a few cysts.
2-2-77: Right upper zone consolidation.
8-2-77: Thin walled cyst at the right base. Complete pneumothorax on the left side [ Fig. 1 ]. This was drained by intercostal tube over the next 2 weeks; the pneumothorax resolved completely but recurred on 2-3-77 and was aspirated.
28-3-77: A few scars in the right middle lobe, small cysts in the right upper lobe, localised empyema anteriorly in the left upper and middle zones with air-fluid level. There was a slow improvement with repeated aspirations.
9-6-77: Small (1 cm) thin walled cyst at the right base. Left lateral pleural thickening in all zones [ Fig. 2 ].
Pulmonary function recorded just before discharge showed: FVC, 1.72 lit; FEV1, 1.49 lit; VC% of predicted, 46%; MEFR, 133.4 lit; PEF, 250 lit; these revealed restriction without any obstruction or improvement after bronchodilator aeresol.
The patient was discharged clinically well on 21-6-77. The recovery was attributed mainly to amphotericin, though gentamicin and penicillin may have helped. He also had a urinary infection with E. coli which responded to ampicillin and mandelamine.
Pulmonary mucormycosis (phycomycosis) was reported as early as 1847.,  The disease is usually a complication of other diseases like diabetes mellitus, malignancy particularly lymphoma, leukemia, tuberculosis, chronic renal and hepatic diseases and extensive burns, administration of steroids, radiation therapy and antimitotic drugs., 
In our patient, chronic otorrhoea treated with various antibiotics was probably the predisposing cause. The fungus might have penetrated through the ear and mastoid cells to cerebrospinal space, meninges and later via blood stream to both the lungs. Our patient had manifestations of systemic toxaemia, meningo- encephalitis and extensive lung-pleural involvement. Due to vascular thrombosis, the lesions are known to produce brawny-red induration representing infarctions. This may happen in various organs leading to fungal dissemination.
The disease may primarily occur in the lungs following the inhalation of the ubiquitous, common atmospheric contaminants. More commonly, however, lung involvement develops as a result of aspiration of infectious materials draining from ulcerative lesions in the paranasal sinuses and nearby mucous membranes or follow haematogenous or lymphogenous dissemination from these sites., 
Pulmonary mucormycosis is of two types. The primary pulmonary form develops from fungal growth in the bronchi. The organism penetrates the bronchial wall and involves hilar tissues. The fungus then invades the arteries and veins leading to thrombosis which results in pulmonary infarctions. Reich and Renzetti suggested that an angiographic demonstration of a thrombosed vessel in an acute pulmonary process in the appropriate clinical setting should raise the possibility of mucormycosis.
The another form causes lobular pneumonia with sudden onset and severe chest pain. Later, there may be pleural friction rub and bloody sputum. Our patient belonged to this form and developed systemic toxemia, empyema, pneumothorax and pleural fibrosis.
By 1960, 57 systemic cases were reported which were oftener in males, McBride et a1 reported that in 32% of cases, brain alone was involved while in 28% cases, lungs were involved and another 28% had a disseminated presentation. In most cases mucor was rarely a primary pathogen. Prior to use of amphotericin B, it has been stated that most cases were fatal. In some pulmonary cases, direct inhalation of spores may be a source.
Though a rhinocerebral form may be fulminant and fatal, the clinical course in our case demonstrated a lack of response to antibiotics and response to amphotericin B as reported by others. Some authors have earlier treated such cases by surgery.,  Spontaneous lysis without any specific therapy of pulmonary mycetoma (fungal balls) due to mucor mycosis, after acute cavitating lung disease has been reported recently.
We wish to thank Dr. P. P. Karnik for reference of the case to our unit. We thank Dr. C. K. Deshpande, Dean for permission to publish the case.