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Elephantine nose due to rhinoentomophthoromycosis

European Journal of Dermatology. Volume 16, Number 1, 87-9, January-February 2006, Clinical report

Summary

Author(s) : Ashok Ghorpade, Podila SA Sarma, Syed Md Iqbal , Department of Dermatology, Venereology & Leprosy, JLN Hospital & Research Centre, Bhilai Steel Plant, Bhilai (Chhattisgarh State), India, Department of General Medicine, JLN Hospital & Research Centre, Bhilai Steel Plant, Bhilai (Chhattisgarh State), India, Department of Otorhinolaryngology, JLN Hospital & Research Centre, Bhilai Steel Plant, Bhilai (Chhattisgarh State), India.

Summary : Rhinoentomophthoromycosis in an immunocompetent Indian male due to Basidiobolus species resulting in a huge (elephantine) nasal deformity, is reported. The diagnosis was done by demonstration of hyphae in direct tissue smear examination in potassium hydroxide, histopathological examination and by cultural characteristics. He showed an excellent response to oral potassium iodide solution.

Keywords : basidiobolomycosis, entomophthoromycosis, potassium iodide, subcutaneous phycomycosis

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Auteur(s) : Ashok Ghorpade¹, Podila SA Sarma², Syed Md Iqbal³

¹Department of Dermatology, Venereology & Leprosy, JLN Hospital & Research Centre, Bhilai Steel Plant, Bhilai (Chhattisgarh State), India
²Department of General Medicine, JLN Hospital & Research Centre, Bhilai Steel Plant, Bhilai (Chhattisgarh State), India
³Department of Otorhinolaryngology, JLN Hospital & Research Centre, Bhilai Steel Plant, Bhilai (Chhattisgarh State), India

accepté le 12 Juillet 2005

Entomophthoromycosis is a progressive subcutaneous granulomatous infection of human beings caused by phycomycetes fungi of the order Entomophthorales [1]. Basidiobolus and Conidiobolus, the common causative agents, normally occur in soil, decaying vegetation and as saprophytes in the gastrointestinal tract of insectivorous animals such as frogs, fish, reptiles, lizards, chameleons, toads and geckoes [2-4]. These get the infection from insects feeding upon their excrement and are in turn eaten by them [3]. Basidiobolomycosis affecting the nose and paranasal region is rare.

Case report

A 37-year-old male, a tailor by occupation, from a village near Bhilai presented with a grotesque swelling over the nose since two years. There was no memory of recent or past trauma. The slowly growing nasal swelling started 2 years earlier, for which a surgeon in a nearby Government hospital had operated upon him one year previously, without any attempt to arrive at a diagnosis. Due to financial constraints, he could attend our hospital only 1 year after surgery. He had developed another swelling on the upper lip six months ago. Due to the facial disfigurement, he remained mostly indoors. Examination revealed a thin built man with a grossly deformed, bulbous nasal swelling, extending onto both cheeks (( figure 1 )). It was firm, mildly tender, skin colored, with normal temperature and a palpable edge, which could be lifted by putting a finger underneath. There were multiple, similar, smaller subcutaneous swellings on his forehead. The mucosal surface of the upper lip had a swelling measuring about 25 mm × 10 mm. Bilateral infraorbital edema was seen. There was no regional adenopathy. Systemic examination was normal. Routine hematology, blood glucose, chest X-ray and CT scan of the paranasal region were normal. Blood tests for HIV 1 and 2 were negative. The subcutaneous tissue from the swellings on the cheeks, upper lip, and the forehead were examined in 10% KOH which showed wide, irregular hyphae with few septa. Histopathology from these lesions revealed multiple dermal granulomas composed of lymphocytes, epitheloid cells, several eosinophils, few foreign body giant cells, and scanty hyphae. A tissue culture on Sabouraud’s dextrose agar at 30 ^oC grew flat, yellowish gray, glabrous colonies within a week, which became radially folded and covered by a fine, powdery white material. Satellite colonies were found, due to germinating conidia ejected from the primary colony. The microscopy of the culture revealed large vegetative hyphae forming round, smooth, thick walled zygospores having closely packed beak like appendages suggesting Basidiobolus species (( figure 2 )). He was put on concentrated potassium iodide solution orally starting with 5 drops (760 mg/ml) diluted with water, once a day and gradually increased to 40 drops thrice daily. There was a dramatic response with appearance of regression of all the swellings in 10 days, and a significant size reduction in 12 weeks (( figure 3 )).

Discussion

Basidiobolomycosis has been reported from Africa, Asia, Europe, North America, South America, and Australia [1, 2, 4]. Joe et al. labeled the fungus “Basidiobolus ranarum” [3]. Less than 15 cases were reported from the Indian subcontinent before 1995 [5, 6]. Basidiobolus species are normal inhabitants of the soil throughout the world. Entrance into the body by minor trauma, insect bites, inhalation or direct inoculation has been postulated [4].

It usually occurs in children, involving the proximal part of extremities, buttocks and the thighs as a firm, subcutaneous, non-tender mass, spreading along contiguous subcutaneous tissue [1, 2]. That the mass can be raised up by inserting the fingers under the edge is supposed to be quite characteristic of this condition [1-3, 5].

Isolation of the same fungal species from multiple consecutive specimens and demonstration of the isolate’s capability to grow at or near the body temperature are required for assessing the infection [7]. In vitro susceptibility testing, as with other fungi, is not reliable in guiding therapeutic decisions [8]. Oral potassium iodide is the drug of choice, and the response to it may be of diagnostic help. Several other drugs including ketoconazole, trimethoprim, sulfamethoxazole, amphotericin, itraconazole and fluconazole have also been used [1, 4-6].

Repeated sub-clinical trauma in our patient, who was a tailor, might have been responsible for acquisition of the infection, manifesting clinically during a state of local or general immunological depression [9]. The classical clinical features, demonstration of fungal hyphae in the direct smear, cultural characteristics and the excellent response to KI helped the diagnosis in our case. The differentiating features of Basidiobolomycosis and Conidiobolomycosis are given in table 1( Table 1 ). Differential diagnosis includes scleroma, rhinosporidiosis, nasal tumors, Burkitt`s lymphoma, soft tissue sarcoma, mycetoma, and fibrosing panniculitis [3-5].

The accompanying lymphatic obstruction in this chronic condition might have contributed to the nasal swelling [7]. Early detection and treatment may help to reduce the morbidity associated with this chronic disease, and prevent disfigurement.
Table 1

Basidiobolomycosis	Conidiobolomycosis
Epidemiology
Tropical regions of East & West Africa, Indonesia, India	Africa, North and South America, South East Asia
Clinical features
* Involves back of shoulders, arms, buttocks, thighs	* Involves nose and paranasal sinuses, forehead, upper lip
* Subcutaneous mass- attached to the skin but not underlying tissue	and periorbital region
* Lymphatic obstruction may cause lymphedema	* Subcutaneous mass - attached to the underlying tissue
Culture
Masses of Zygospores (20-50 µ in diameter) with a prominent beak and chlamydoconidia in addition to sporangia are observed.	Sporangiola are single celled; spores often covered with short hair like appendages called villae; may be ejected from the sporangiola and can travel a distance of 30 mm.
Histopathology
*An inflammatory reaction with lymphocytes, plasma cells, histiocytes and giant cells.	Similar histopathology
* Broad , thin walled hyphae with occasional septae, branched at right angles and fibroblastic proliferation.
* Splendore-Hoeppli phenomenon may be seen.

Acknowledgements

The authors are thankful to Dr. Mrs. S. Mohanty for fungal culture and Dr. C. Ramanan, Dr. M. N. Das, Dr. P. Mercy, Dr.A.K Garg, and Dr. M. Bhalla for their technical help.

References

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4 Sugar AM. Agents of mucormycosis and related species. In: Mandell GL, Bennett GE, Dolin R editors, Mandell, Douglas, and Bennett`s, eds. Principles and Practice of Infectious Diseases, 5^thed. NewYork: Churchill: Livingstone, 2000: 2685-95.

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