(1975), 13, 44-48




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Department of Dermatology, Madras Medical College and Government General Hospital, Madras, India. Two cases of Basidiobolomycosiscaused by species of the order Entomophthorales are discussed with the report on lymph node involvement by B. haptosporus.A clearer aetiological classification of the diseases caused by various agents belonging to the class Phycomycetesis preferred in view of the distinct clinical entities produced by them. Involvement of the lymph nodes is also an evidence that the disease produced by B. haptosporusis not restricted to subcutaneous tissue. A routine lymph node biopsywith mycologicalstudies of the node is suggested in such cases. The first case of subcutaneous phycomycosis was reported from Indonesia by Lie-Kian-Joe, Njo, Pohan, Van der Meulen & Emmons (1956). The agent identified as Basidiobolus ranarum in this case was later shown to be B. haptosporus (Emmons, Binford & Utz, 1970). Since then there have been many reports (Harman, Hughlings Jackson & Willis, 1964; Angela Restrepo, Greer, Mario Robledo, Constanza Diaz, Roberto Lopez & Cesar Bravo, 1967; Vanbreuseghem, 1970) including India (Koshi, Kurien, Sudarsanam, Selvapandian & Mammen, 1972; K a m a l a m & Thambiah, 1972; Kamalam, Yesudian & Thambiah, 1973). Follow up of these patients with or without treatment extended from a few days to 3 years. In none of the reported cases was there any proved fungal invasion of lymph nodes. This paper reports involvement of lymph nodes by B. haptosporus in one of 2 cases reported earlier (Kamalam, et al., 1973).

Case 1 In 1968, a 40 year old female reported at the Government General Hospital, Madras with plaques over the nose, cheeks and forehead of 11 months duration. The case was diagnosed as subcutaneous phycomycosis by the presence of an eosinophilic granuloma and coenocytic hyphae in tissue sections. Culture was not obtained. Her left submandibular lymph nodes were enlarged, discrete and nontender (fig. 1). Permission for a biopsy of the lymph node was not obtained. Treatment consisted of 3.6 g of oral K I daily for 6 months followed by surgical excision. With much improvement of her skin condition she was discharged 18 months after admission. One year later she came back with perforation of the hard palate and difficulty in swallowing. There was considerable reduction in body weight and the original lymphadenopathy was still present. As she refused in-patient treatment, oral K I in the same dosage was advised. She has not returned but we were informed that she is alive.

Case 2 A 35 year old male reported to us in May 1972 with an ulcerated painful plaque over and around the right thigh of 6 months duration. The right inguinal lymph nodes were enlarged, firm, tender and freely mobile. An initial clinical diagnosis of subcutaneous phycomycosis was confirmed as Basidiobolomycosis by cultural and histological studies. Lymph node biopy was refused. He was treated with oral K I (3.6 g daily) for 3 months. During the 3rd month of therapy he developed pitting oedema of the affected limb which resolved with diuretics. This associated lymphatic system involvement was thought to be benign and temporary. He was advised to 44

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continue K I and sent home with almost a complete resolution of the skin lesions and much reduction in size of the lymph nodes (fig. 2). He returned in March 1973 with recurrence of skin lesions at the original site with enlargement of femoral and inguinal lymph nodes and mild oedema of the right leg. Recurrence was attributed to the discontinuation of therapy after J a n u a r y for 2 months. K I was reinstituted as outpatient treatment. He returned for the 3rd time in October 1973 with reappearance of a plaque (4 cm x 6 cm) over the posterior side of the right thigh. The lymph nodes in the original site were much enlarged, adherent to one another and tender, with another single lymph node on the right inguinal fold. One month prior to returning to hospital he had discontinued K I . This time he permitted a biopsy of the right inguinal lymph node. Macroscopically it was irregular in shape and firm in certain areas. Sections showed a pale surface with abscesses containing odourless brown pus. K O H preparation of the pus showed broad septate hyphae and round bodies with a beak resembling conid~a. Some of the hyphae were found to be surrounded by granular material. Culture of the lymph node on Sabouraud's dextrose agar revealed typical colonies of Basidiobolus sp. in 3 days. Microscopically it showed the characteristic features of B. haptosporus similar to the culture obtained from the skin tissue during first admission. Sections of the lymph node were stained with H & E, PAS and Gomori's methenamine silver.

Histology of the Lymph Node H & E sections showed a very disorganised gland, distortion of follicular architecture with islets of pale staining areas and cell debris with erythrocytes, collections of deep staining cells, giant cells of foreign body and, Langhans types, eosinophils, hlstiocytes and plasma cells. Vessels in the cell collection were engorged. Fatty tissue surrounding the glandular mass was also permeated by massive cell infiltrate and islets of pale staining areas of cell debris surrounded by deeply stained cells, eosinophils, histiocytes and giant cells (foreign body and Langhans types).

Figure 1.--Case 1 shows enlarged left submandibular lymph nodes. Figure 2.--Case 2 shows complete resolution of the skin lesions and reduction in size of lymph nodes of right thigh. Figure 3.--Lymph node (case 2) section in GMS stain shows the hyphal structures of B. haptosporus scattered throughout ( × 320).

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A. K A M A L A M A N D A. S. T H A M B I A H

Vessels in these areas were also engorged and vessels in the gland and surrounding tissue were not invaded by the fungus. PAS stain showed evidence of fungal structure throughout the infiltrate. There was no clear evidence of vessel invasion in the sections studied. GMS stain: This confirmed the presence of hyphae scattered among the cell debris and even inside giant cells (fig. 3). Evidence of the fungus was also seen in the extraglandular fatty tissue and near the capsule. K I was reinstituted (3"6 g daily) and the patient was discharged after a month and advised to continue the drug as he was not willing for a total lymph node excision. His skin lesions had completely disappeared and lymph nodes regressed considerably during the 2 months period. He is still being followed up for further developments. Amphotericin B. was not available for this patient. DISCUSSION Though the disease is called subcutaneous phycomycosis, there have been reports of deeper tissue involvement such as the muscles, by B. haptosporus, (Vanbreuseghem, 1970; Koshi et al., 1972). Vanbreuseghem (1970) reported paralysis and atrophy of the muscles of the affected limb and failure of the mucsles to react to electrical stimulation. Among the cases reported by Harman et al., (1964) 1 showed enlarged femoral and inguinal lymph nodes and another case showed a splenic enlargement of 8 cm below the costal margin. The lymph node in the former case showed reactive hyperplasia. Koshi et al., (1972) made no particular mention of lymph node enlargement but reported that there was non-specific follicular hyperplasia without fungal elements. Vanbreuseghem (1970) had observed sinuses in the axilla of his patient during a 2 year follow up and B. meristosporus was cultured from the discharge. This finding might have been due to a lymph node, affected by the fangus, breaking down to form a sinus resembling that in tuberculous scrofuloderma. L y m p h node invasion by this fungus has been referred to by Ridley & Wise (1965), Edington (1964), Villasco, Camain, Mazere & Segretain (1966) and Martinson & Clark (1967). However, to our knowledge our 2nd case is the first report in the literature where such involvement by B. haptosporus has been proved by culture and histologicy. Whether the lymph node was previously affected, by an organism like Mycobacterium tuberculosus was thought of but histological studies with H & E and Ziehl Neelsen stains showed no such involvement. No blood vessel invasion by the fungus was ob:erved in the several sections studied, though erythrocytes in the cell debris denoted vessel damage. Though culture was not obtained in our Ist case, on review, a diagnosis of Entomophthoromycosis could be entertained when one considers the sites affected. The fungal origin of the enlarged lymph nodes in this case could not be proved as the patient did not allow a biopsy. Perforation of the palate is evidence that the fungus could extend deeper than submucous tissues. A sldagram of facial bones was not sanctioned. Occasional involvement of pharynx, large intestine, abdominal organs and pelvic organs is suggested to be possible (Emmons et al., 1970). These findings and the present report of our cases reveal that subcutaneous phycomycosis is not restricted to the subcutaneous fat but definitely extends to deeper tissues like muscles and lymph nodes. As the disease has been reported only for 18 years, one has to keep vigilant about the extent of the disease, prognosis and therapy. As the follow up of the cases reported already by various authors were for only a few years, it is too early

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to decide a b o u t the prognosis or cure of the disease with the a v a i l a b l e m e t h o d s o f therapy. But, w h a t is evident is t h a t this disease is not fatal a n d n o t associated w i t h a n y p r i m a r y d e b i l i t a t i n g illness such as diabetes mellitus as with infections caused b y Mucor sp. I n c o n t r a s t to t h a t fungus B. haptosporus is not k n o w n to i n v a d e the blood vessels a n d runs a benign chronic course. T h o u g h o u r observations were o n l y on 2 cases, we suggest t h a t cases d i a g n o s e d as subcutaneous phycomycosis should always be investigated w i t h the regional l y m p h node biopsy for e a r l y detection of invasion b y the fungus. Cases should also be followed up with or w i t h o u t t r e a t m e n t for a long time. W e agree w i t h the view given b y V a n b r e u s e g h e m (1970) a n d prefer the following n a m e s : 1. Basidiobolomycosis, for a n infection caused b y B. haptosporus; 2. Entom o p h t h o r o m y c o s i s , for infection caused b y a n E n t o m o p h t h o r a l e such as E. coronata a n d 3. M u c o r m y c o s i s , for infections caused b y M u c o r a l e s i.e. Rhizopus, Absidia & Mortierella; A 4 t h s e p a r a t e grouping, Phycomycosis, for infection caused b y a fungus of the class P h y c o m y c e t e s where the organisms do not c o m e u n d e r the above 3 groups or has not b e e n identified b y culture b u t d i a g n o s e d on histilogical section as suggested by E m m o n s et al., (1970). W e feel t h a t this suggestion is logical as the clinical entities p r o d u c e d b y these 4 groups of fungi a r e distinct. T h e n a m e subcutaneous p h y c o m y cosis is confusing as the fungus can involve d e e p e r structures like l y m p h nodes as in the present r e p o r t a n d m a y therefore be p o t e n t i a l l y systemic. ZUSAMMENFASSUNG

Zwei Fiille einer Basidiobolomycosis, die durch Entomophthorales-Arten verursacht wurden, werden mit dem Befund tiber eine Lymphknotenbeteiligung durch B. haptosporus diskutiert. Eine genauere /itiologische Klassifizierung der Krankheiten, die durch verschiedene zur Klasse der Phycomycetes z/ihlenden Erreger hervorgerufen werden, ist im Hinblick auf die dutch sic verursachten eindeutigen klinischen Krankheitsbilder anzustreben. Die Mitbeteiligung der Lymphknoten ist ein Beweis daftir, da .~ die durch B. haptosporus ausgel6ste Krankheit nicht auf das subcutane Gewebe beschriinkt ist. In solchen FAllen wird eine routinemti [31g zu erfolgende Biopsie mit mykologischer Untersuchung des Lymphknctens empfohlen. REFERENCES RESTREeO, Angela M., G~EER, D. L., ROBLEDO,M. V., CONSTANZADIAZ,G., LoPEz, R. M. & BRAVO, C. R. (1967). Subcutaneous phycomycosis: Report of first case observed in Colombia, South America. American Journal of Tropical Medicine, 16, 34-39. EmNGTON, G. M. (1964). Phycomycosis in Ibadan, Western Nigeria. Two postmortem reports. Tramactions of the Royal Society of Tropical Medicine and Hygiene, 5B, 242-245. EMMONS,C. W., Binford, C. H. & UTZ, J. P. (1970). Medical Mycology, 2nd Edition, Lea & Febiger, Philadelphia, 230-255. Kosm, Grace, THANKAMMA,Kurien, SUDARSANAM,D., SELVAPANDIAN,A. J. & MAMMEN, K. E. (1972). Subcutaneous phycomycosis caused by Basidiobolus. A report of three cases. Sabouraudia, 10, 237-243. HARMAN,R. R. M., HUGHLINGS,J. ~: WILLIS,A . J . P . (1964). Subcutaneous phycomycosis in Nigeria. British Journal of Dermatology, 76, 408-420. KAMALAM,A. ~: THAMBIAH,A. S. (1972). Subcutaneous phycomycosis in Madras. Report of a case treated medically and surgically. Antiseptic, 69, 437-441. KAMALAM,A., YESUDIAN,P. • THAMBIAH,A. S. (1973). Basidiobolomycosis - A case report. The AustralasionJournal of Dermatology,XIV, 136-139. LIE-KIAN-JoE, NjO-INjo-TjoEI ENG, POHAN, A., VAN DER MEULEN, A. & EMMONS, C. W. (1956). Basidiobolus ranarumas a cause of subcutaneous mycosis in Indonesia. A.M.A., Archivesof Dermatology, 74, 378-383. MARTINSON,F. D. & CLARK,Betty M. (1967). Rhinophycomycosis entomophthorae in Nigeria. American Journal of Tropical Medicine and Hygiene, 16, 4047. RXDL~Y, D. S. & WISE, M. (1965). Unusual disseminated infection with a phycomycete. Journal of Pathologyand Bacteriology,90, 675-679.



VANBREUSIEGHAM,R, (1970). Evolution a n d t r e a t m e n t o f a case of Basidiobolomycosis. I1. International

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Congress of Infectious Diseases. Vienna, 13-16. VXLLASCO, J., CAUAIN, R., MAZ~RE, J. & SEGRETAIN, G. (1966). Isol6ment d ' u n deuzieme cas de phycomycose en c6te d'Ivoire avec isoMment de la souche. Bulletin de la Soci6tg de Pathologie 6xotique, 59, 781-786.

Basidiobolomycosis with lymph node involvement.

Two cases of Basidiobolomycosis caused by species of the order Entomophthorales are discussed with the report on lymph node involvement by B. haptosph...
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