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| Year : 2016 | Volume
: 61
| Issue : 6 | Page : 697-699 |
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| An old woman with a lump |
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Pooja Arora1, Kabir Sardana2, Anjali Madan2, Nita Khurana3
1 Department of Dermatology, Dr. Ram Manohar Lohia Hospital and PGIMER, New Delhi, India
2 Department of Dermatology, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India
3 Department of Pathology, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India
| Date of Web Publication | 9-Nov-2016 |
Correspondence Address:
Pooja Arora
9547, Sector C, Pocket 9, Vasant Kunj, New Delhi - 110 070
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0019-5154.193705
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How to cite this article:
Arora P, Sardana K, Madan A, Khurana N. An old woman with a lump. Indian J Dermatol 2016;61:697-9 |
A 60-year-old female, resident of Bihar, presented to our outpatient department with a gradually progressive asymptomatic swelling over the lower half of face for the past 20 years [Figure 1]. There was thickening of the lower lip and difficulty in opening the mouth for the past 2 years. Her past medical history was unremarkable. Examination revealed a subcutaneous hard swelling involving the perioral region extending on to the submandibular area. The skin overlying the swelling was erythematous. There was associated thickening of the lower lip. Lymphadenopathy was absent. The hematoxylin and eosin-stained section of biopsy specimen [Figure 2]a and b revealed granulomatous inflammation in the lower dermis. The granuloma consisted predominantly of histiocytes along with plasma cells, eosinophils, and few giant cells. Culture for fungus was negative. High-resolution computed tomography scan of the paranasal sinuses showed mucosal thickening in the bilateral maxillary sinus and sphenoid sinus with mild atrophy of bilateral middle turbinates. Hematological and biochemical parameters were normal. | Figure 1: Erythematous hard swelling over the lower half of face with thickening of the lower lip
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 | Figure 2: (a) Granulomatous inflammation in the lower dermis (H and E, ×40). (b) Multinucleated giant cells with well-defined tubular structures inside the giant cells (H and E, ×400)
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 Question | |  |
What is your diagnosis?
| Answer | | |
 View Answer
Diagnosis - Subcutaneous zygomycosis (rhinofacial conidiobolomycosis).
| Discussion | | |
Among the zygomycetes, two orders are of medical importance - the Mucorales and the Entomophthorales. Mucorales comprises Mucor and Rhizopus spp. and causes mucormycosis which is an acute angioinvasive infection occurring in immunocompromised patients. Entomophthorales has three pathogenic species: Basidiobolus ranarum, Conidiobolus coronatus, and Conidiobolus incongruous.[1] These fungi can be isolated from soil and decaying plant matter. Infection occurs by traumatic inoculation or inhalation. Cutaneous infection causes woody hard subcutaneous swellings that do not pit, spread locally, and rarely cause disseminated disease. [2] Entomophthoromycosis usually occurs in immunocompetent patients. Basidiobolomycosis tends to affect the limb girdle region and buttocks in children, whereas conidiobolomycosis affects the rhinofacial region in adult males. [3] The latter is eight times more common in males.
Out of the zygomycetes, C. coronatus is the species that infects nasal mucosa and submucosa and spreads to the adjacent tissues, especially the paranasal sinuses, nose, upper lip, palate, pharynx, cheek. Rarely, deep infection has been reported. Due to this site of involvement, infection with C. coronatus is also known as rhinoentomophthoromycosis. This patient also had involvement of the nose and paranasal sinuses with atrophy of the turbinates.
Direct microscopy of the teased biopsy specimen in 10% potassium hydroxide may be done. Histopathological examination shows a suppurative granuloma with predominantly eosinophils apart from lymphocytes, plasma cells, histiocytes, and multinucleated giant cells. Dense fibrosis may be seen. Fungus can be identified as nonseptate hyphae within the multinucleated giant cells using hemotoxylin and eosin stain, periodic acid-Schiff stain, or Gomori-Grocott stain [Figure 3]. [4] Hyphae may appear ring-shaped or oval in cross-sections. Splendore-Hoeppli phenomenon (hyphae surrounded by an eosinophilic halo) is the characteristic histopathological feature. Fungus can be isolated in Sabouraud dextrose agar (without cycloheximide or chloramphenicol) at 30°C-37°C. Colonies appear within 4 days as white or brown with a grainy look and irregular borders. Examination of culture shows a hyaline mycelium with few or no septum. Primary conidia are seen as spherical drop shaped with a prominent papilla. The fungus is rarely isolated as the large coenocytic hyphae of zygomycetes can get damaged during biopsy procedure resulting in a negative fungal culture. [5],[6] | Figure 3: Broad aseptate hyphae with scanty cytoplasm appearing as tube-like structures seen within fungal granulomas (Methenamine silver stain, ×1000)
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The differential diagnosis includes lymphoma, sarcoma, lupus profundus, and sarcoidosis.
Potassium iodide, ketoconazole (400 mg/day), itraconazole (300 mg/day), or fluconazole (300 mg/day), amphotericin B (0.5 mg/day), dapsone (1-2 mg/kg of body weight daily), terbinafine are the treatment options available. [7],[8] Cotrimoxazole can be added to the treatment for additional benefit.
We chose to treat our patient with saturated solution of potassium iodine in gradually increasing doses. Baseline thyroid function test and serum potassium levels were done before starting treatment. The maximum dose reached was thirty drops three times a day. The patient started showing response after 2 weeks of initiating treatment. Woody hard swelling became soft and there was improvement in mouth opening. The swelling resolved completely in 8 weeks. The treatment was continued for 12 weeks. No side effects were seen during treatment. There were no deformities or recurrence at 1 year of follow-up.
Learning points
- Conidiobolomycosis usually affects the rhinofacial region in immunocompetent adults
- The infection is characterized clinically by woody hard subcutaneous swellings that spread locally to involve the nose and paranasal sinuses
- Biopsy shows suppurative granulomas with multinucleated giant cells and fungal hyphae surrounded by eosinophilic sheath
- Fungal culture may be negative as the hyphae are fragile
- Itraconazole, ketoconazole, terbinafine, amphotericin, and potassium iodide are the treatment options available.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Williams AO. Pathology of phycomycosis due to Entomophthora and Basidiobolus species. Arch Pathol 1969;87:13-20.  |
| 2. | Herstoff JK, Bogaars H, McDonald CJ. Rhinophycomycosis entomophthorae. Arch Dermatol 1978;114:1674-8. |
| 3. | Hay RJ, Ashbee HR. Mycology. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 8 th ed. Oxford: Blackwell Science Ltd.; 2004. p. 36.79. |
| 4. | Gilbert EF, Khoury GH, Pore RS. Histopathological identification of Entomophthora phycomycosis. Deep mycotic infection in an infant. Arch Pathol 1970;90:583-7. |
| 5. | Thappa DM, Kartikeyan K, Sujatha S. Subcutaneous zygomycosis: Current Indian scenario with a review. Indian J Dermatol 2003;48:212-8.  |
| 6. | Arora P, Sardana K, Bansal S, Garg VK, Rao S. Entomophthoromycosis (Basidiobolomycosis) presenting with saxophone penis and responding to potassium iodide. Indian J Dermatol Venereol Leprol 2015;81:616-8. [ PUBMED] |
| 7. | Krishnan SG, Sentamilselvi G, Kamalam A, Das KA, Janaki C. Entomophthoromycosis in India - A 4-year study. Mycoses 1998;41:55-8. |
| 8. | Isa-Isa R, Arenas R, FernIsa R RF, Isa M. Rhinofacial conidiobolomycosis (entomophthoramycosis). Clin Dermatol 2012;30:409-12. |
[Figure 1], [Figure 2], [Figure 3] |
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