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Oral submucous fibrosis in a Greek female
0007-l 17X/81/00320197~02.00
British Journal of Oral Surgery (1981) 19, 197-201 @ The British Association
of Oral Surgeons
ORAL SUBMUCOUS FIBROSIS IN A GREEK FEMALE GEORGE LASKARIS,D.D.S., M.D.,OLGA BOVOPOULOU, D.D.S. and GEORGE NICOLIS, M.D. The University of Athens Department of OralPathology, School of Dentistry and Department of Dermatology, ‘A. Sygros’ Hospital, School of Medicine, 20-22 Ipsilandou Street, Athens 140, Greece
Summary. Submucous fibrosis of the oral cavity is a disease affecting almost exclusively the Indian race, although sporadic cases have been described in non-Indians. Its aetiology is unknown and dietary factors have been incriminated in its pathogenesis, such as chilli ingestion. In this paper we present a case of oral submucous fibrosis in a 67-year-old Greek female, who two years after initial diagnosis developed squamous cell carcinoma of the tongue. The clinical course and histological findings are described and the potential of this disease as a predisposing factor for the development of malignancies of the oral mucosa is discussed.
Introduction S&mucous fibrosis of the oral cavity was first described by Schwartz (1952) in five Indian females from Kenya and East Africa. Initially the term ‘atrophia idiopathica mucosae oris’ was proposed (Schwartz, 1952), which was later replaced by the term currently being used (Joshi, 1953; Lal, 1953). Submucous fibrosis has been described almost exclusively in Indians. The prevalence in India rages from 0.2 to 0.5 per cent with a higher predominance in the southern parts of the Indian subcontinent (Pindborg & Sirsat, 1966). Individuals between the ages of 20 and 40 years are most commonly affected (Kennedy & MacDonald, 1968) but cases have been reported in patients ranging from two to 89 years of age. Several mechanisms have been proposed for the pathogenesis of the disease, the most favourable being the ‘collagen disease’ hypothesis according to which submucous fibrosis is considered a variant of scleroderma, and the ‘allergic response’ hypothesis which suggests that the disease is an altered inflammatory response to the ingestion of spices and especially chillis (Hammer et al., 1971; Binnie and Cawson, 1972). So far only a handful of cases have been described in Caucasians. In this paper a case of oral submucous fibrosis is presented in an elderly Greek female who later developed squamous cell carcinoma of the tongue which progressed to a fatal termination. Case report A 67-year-old Greek female, who was born in Russia and had resided there for the first 20 years of her life, came to ‘Andreas Sygros’ Hospital complaining of a burning sensation in the mouth for the previous one and a half years. On clinical examination the oral mucosa was pale and atrophic while the tongue was smooth with papillary atrophy, slightly enlarged and stiff (Fig. 1). The buccal mucosa and lower lip were smooth and pale while the hard palate was reddish and interspersed with vesicles. On palpation the oral mucosa was inelastic and extremely tender. Trismus was present. (Received 5 March 1980; accepted 10 July 1980)
197
BRITISH
198
JOURNAL
OF ORAL
SURGERY
FIG. 1. Severe atrophy of tongue papillae.
The past medical history of the patient revealed rheumatic disease, rheumatic mitral stenosis and a myocardial infarction that had occurred three years previously. For her cardiac problems, the patient was on a digitalis preparation and the diuretic frusemide. Routine laboratory tests (Table I) showed a Hb of 13 gm/lOO dl, Hct 41x, WBC 7,40O/mms with 72 % polymorphs and 28 % lymphocytes, an ESR of 70 mm per hour and normal urinalysis. Serological tests for syphilis were negative. A biopsy specimen from the dorsum of the tongue was obtained, stained with haematoxylin and eosin and viewed under a light microscope. Epithelial atrophy, disappearance of the rete ridges and hyaline degeneration of the juxta-epithelial collagen bundles extending to the deeper layers of the thorium were observed (Fig. 2). No epithelial dysplasia was noted. The patient was placed on 20 mg prednisone per day for one month and then the dosage of the steroid was tapered off and discontinued. Six months later the stiffness of the tongue, the burning sensation and trismus had worsened. A slight improvement was achieved with 10 mg prednisone per day and 4 gm of vitamin C for over four months. However, two years later, she returned with even worse symptomatology and at this time two ulcerations with indurated margins and granular surfaces were Table I
Results of the Laboratory
Investigations
Hb
Hct
WBC
ESR
Urinalysis
Serologic tests for syphilis
13 gm
41%
74OO/mm3
70 mm
normal
negative
Histopathology Epithelial atrophy, hyaline degeneration of the thorium, no epithelial dysplasia
ORAL
SUBMUCOUS
FIBROSIS
IN
A GREEK
FEMALE
199
FIG. 2. Epithelial atrophy, loss of rete peg structure, hyperkeratosis and moderate hyalinization of the connective tissue (Haematoxylin-eosin stain. x 155).
FIG. 3. Two independent
squamous-cell carcinomas (arrows) on the tongue of the patient which developed two years later.
200
BRITISH
JOURNAL
OF
ORAL
SURGERY
observed on the dorsal aspect of the tongue (Fig. 3). Enlargement of the regional nodes was detected and biopsy from the ulcers showed squamous cell carcinoma. Surgical excision was carried out but the patient died six months post-operatively, due to widespread metastases. Discussion Submucous fibrosis of the oral cavity is a chronic disease of unknown aetiology that is encountered almost exclusively in Indians. Three cases have been described in Chinese of Taiwan, and a few more in Europeans residing in Hyderabad (Rao, 1962). Simpson (1969) has also described a case in an English woman married to a Pakistani for 12 years and being under a normal Indian diet. The disease has also been reported to affect Indian immigrants who had preserved their traditional dietary habits and use spices and chillis. The latter have been incriminated along with betel nuts in the pathogenesis of the disease (Hammer et al., 1971; Binnie & Cawson, 1972). Several unusual features are noted in the case presented in this report. The disease occurred in a Greek female who have never been in India, denied chilli ingestion and had the dietary habits of the average Greek which are totally different from those of the Indian population. The patient did not drink, smoke, or use other irritants of the oral mucosa. Although a number of patients with oral submucous fibrosis have an increased ESR (Simpson, 1969), in our case the high ESR might have been attributed to the rheumatic disease which is mentioned in the patient’s past medical history. A serious problem in our case was the final diagnosis. Several diseases and conditions (e.g. sideropenic anaemia, scleroderma, discoid lupus erythematosus, epidermolysis bullosae, dyskeratosis congenita, lipoid proteinosis) which cause atrophy and underlying fibrosis of the oral mucosa and, finally, predispose to the development of squamous cell carcinoma (Pindborg, 1980) should be considered in the differential diagnosis. Nevertheless, the clinical and laboratory findings in our case did not advocate towards the above-mentioned diseases. The past medical history, the clinical features and the laboratory findings support the view that the most likely diagnosis in our case is oral submucous fibrosis. The development of squamous cell carcinoma at an advanced stage of the disease supports the view of other investigators that this entity represents a precancerous state. Pindborg (1972) in an extensive study showed that epithelial atypia existed in 13.2 per cent of all histological specimens from the oral mucosa of patients with oral submucous fibrosis. Furthermore, the same author (Pindborg et al., 1964) reports an incidence of leukoplakia in 28 per cent of patients affected with submucous fibrosis, which is nine fold higher than the control population. References Binnie, W. H. & Cawson, R. A. (1972). A new ultrastructural British
Journal of Dermatology,
finding in oral submucous
fibrosis.
86, 286.
Hammer, J. E. III, Mehta, F. S., Pindborg, J. J. & Daftary, D. K. (1971). Altered staining reaction of connective tissues in 53 submucous fibrosis patients. Journal of Dental Research, 50, 388. Joshi, S. G. (1953). Submucous fibrosis of the palate and pillars. Indian Journalof Otolaryngology, 4,1. Kennedy, T. F. & MacDonald, D. G. (1968). Oral submucous fibrosis. A case report. British Dental Journal, 124, 121. Lal, D. (1953). Diffuse oral submucous fibrosis. Journal of All-Zndia Dental Association, 26, 1. Pindborg, J. J., Chawla, T. N., Srivastava, A. N., Gupta, D. & Mehrotra, M. L. (1964). Clinical aspects of oral submucous fibrosis. Acta Odontologieal Scandinavica, 22, 679. Pindborg, J. J. & Sirsat, S. M. (1966). Oral submucous fibrosis. Oral Surgery, Oral Medicine and Oral Pathology,
22, 764.
ORAL
SUBMUCOUS
FIBROSIS
IN
Pindborg, J. J. (1972). Is submucous fibrosis a precancerous Dental Journal, 22, 474. Pindborg, J. J. (1980). Oral Cancer and Precancer.
A
GREEK
FEMALE
201
condition in the oral cavity? Znternntional
Bristol: Wright. Rao, A. B. N. (1962). Idiopathic palatal fibrosis. British Journal of Surgery, 50, 23. Schwartz, J. (1952). Atrophic idiopathica (tropica) mucosaeoris. Demonstrated at the 11th International Dental Congress, London. Simpson, W. (1969). Submucous fibrosis. British Journal of Oral Surgery, 6, 196.
British Journal of Oral Surgery (1981) 19, 197-201 @ The British Association
of Oral Surgeons
ORAL SUBMUCOUS FIBROSIS IN A GREEK FEMALE GEORGE LASKARIS,D.D.S., M.D.,OLGA BOVOPOULOU, D.D.S. and GEORGE NICOLIS, M.D. The University of Athens Department of OralPathology, School of Dentistry and Department of Dermatology, ‘A. Sygros’ Hospital, School of Medicine, 20-22 Ipsilandou Street, Athens 140, Greece
Summary. Submucous fibrosis of the oral cavity is a disease affecting almost exclusively the Indian race, although sporadic cases have been described in non-Indians. Its aetiology is unknown and dietary factors have been incriminated in its pathogenesis, such as chilli ingestion. In this paper we present a case of oral submucous fibrosis in a 67-year-old Greek female, who two years after initial diagnosis developed squamous cell carcinoma of the tongue. The clinical course and histological findings are described and the potential of this disease as a predisposing factor for the development of malignancies of the oral mucosa is discussed.
Introduction S&mucous fibrosis of the oral cavity was first described by Schwartz (1952) in five Indian females from Kenya and East Africa. Initially the term ‘atrophia idiopathica mucosae oris’ was proposed (Schwartz, 1952), which was later replaced by the term currently being used (Joshi, 1953; Lal, 1953). Submucous fibrosis has been described almost exclusively in Indians. The prevalence in India rages from 0.2 to 0.5 per cent with a higher predominance in the southern parts of the Indian subcontinent (Pindborg & Sirsat, 1966). Individuals between the ages of 20 and 40 years are most commonly affected (Kennedy & MacDonald, 1968) but cases have been reported in patients ranging from two to 89 years of age. Several mechanisms have been proposed for the pathogenesis of the disease, the most favourable being the ‘collagen disease’ hypothesis according to which submucous fibrosis is considered a variant of scleroderma, and the ‘allergic response’ hypothesis which suggests that the disease is an altered inflammatory response to the ingestion of spices and especially chillis (Hammer et al., 1971; Binnie and Cawson, 1972). So far only a handful of cases have been described in Caucasians. In this paper a case of oral submucous fibrosis is presented in an elderly Greek female who later developed squamous cell carcinoma of the tongue which progressed to a fatal termination. Case report A 67-year-old Greek female, who was born in Russia and had resided there for the first 20 years of her life, came to ‘Andreas Sygros’ Hospital complaining of a burning sensation in the mouth for the previous one and a half years. On clinical examination the oral mucosa was pale and atrophic while the tongue was smooth with papillary atrophy, slightly enlarged and stiff (Fig. 1). The buccal mucosa and lower lip were smooth and pale while the hard palate was reddish and interspersed with vesicles. On palpation the oral mucosa was inelastic and extremely tender. Trismus was present. (Received 5 March 1980; accepted 10 July 1980)
197
BRITISH
198
JOURNAL
OF ORAL
SURGERY
FIG. 1. Severe atrophy of tongue papillae.
The past medical history of the patient revealed rheumatic disease, rheumatic mitral stenosis and a myocardial infarction that had occurred three years previously. For her cardiac problems, the patient was on a digitalis preparation and the diuretic frusemide. Routine laboratory tests (Table I) showed a Hb of 13 gm/lOO dl, Hct 41x, WBC 7,40O/mms with 72 % polymorphs and 28 % lymphocytes, an ESR of 70 mm per hour and normal urinalysis. Serological tests for syphilis were negative. A biopsy specimen from the dorsum of the tongue was obtained, stained with haematoxylin and eosin and viewed under a light microscope. Epithelial atrophy, disappearance of the rete ridges and hyaline degeneration of the juxta-epithelial collagen bundles extending to the deeper layers of the thorium were observed (Fig. 2). No epithelial dysplasia was noted. The patient was placed on 20 mg prednisone per day for one month and then the dosage of the steroid was tapered off and discontinued. Six months later the stiffness of the tongue, the burning sensation and trismus had worsened. A slight improvement was achieved with 10 mg prednisone per day and 4 gm of vitamin C for over four months. However, two years later, she returned with even worse symptomatology and at this time two ulcerations with indurated margins and granular surfaces were Table I
Results of the Laboratory
Investigations
Hb
Hct
WBC
ESR
Urinalysis
Serologic tests for syphilis
13 gm
41%
74OO/mm3
70 mm
normal
negative
Histopathology Epithelial atrophy, hyaline degeneration of the thorium, no epithelial dysplasia
ORAL
SUBMUCOUS
FIBROSIS
IN
A GREEK
FEMALE
199
FIG. 2. Epithelial atrophy, loss of rete peg structure, hyperkeratosis and moderate hyalinization of the connective tissue (Haematoxylin-eosin stain. x 155).
FIG. 3. Two independent
squamous-cell carcinomas (arrows) on the tongue of the patient which developed two years later.
200
BRITISH
JOURNAL
OF
ORAL
SURGERY
observed on the dorsal aspect of the tongue (Fig. 3). Enlargement of the regional nodes was detected and biopsy from the ulcers showed squamous cell carcinoma. Surgical excision was carried out but the patient died six months post-operatively, due to widespread metastases. Discussion Submucous fibrosis of the oral cavity is a chronic disease of unknown aetiology that is encountered almost exclusively in Indians. Three cases have been described in Chinese of Taiwan, and a few more in Europeans residing in Hyderabad (Rao, 1962). Simpson (1969) has also described a case in an English woman married to a Pakistani for 12 years and being under a normal Indian diet. The disease has also been reported to affect Indian immigrants who had preserved their traditional dietary habits and use spices and chillis. The latter have been incriminated along with betel nuts in the pathogenesis of the disease (Hammer et al., 1971; Binnie & Cawson, 1972). Several unusual features are noted in the case presented in this report. The disease occurred in a Greek female who have never been in India, denied chilli ingestion and had the dietary habits of the average Greek which are totally different from those of the Indian population. The patient did not drink, smoke, or use other irritants of the oral mucosa. Although a number of patients with oral submucous fibrosis have an increased ESR (Simpson, 1969), in our case the high ESR might have been attributed to the rheumatic disease which is mentioned in the patient’s past medical history. A serious problem in our case was the final diagnosis. Several diseases and conditions (e.g. sideropenic anaemia, scleroderma, discoid lupus erythematosus, epidermolysis bullosae, dyskeratosis congenita, lipoid proteinosis) which cause atrophy and underlying fibrosis of the oral mucosa and, finally, predispose to the development of squamous cell carcinoma (Pindborg, 1980) should be considered in the differential diagnosis. Nevertheless, the clinical and laboratory findings in our case did not advocate towards the above-mentioned diseases. The past medical history, the clinical features and the laboratory findings support the view that the most likely diagnosis in our case is oral submucous fibrosis. The development of squamous cell carcinoma at an advanced stage of the disease supports the view of other investigators that this entity represents a precancerous state. Pindborg (1972) in an extensive study showed that epithelial atypia existed in 13.2 per cent of all histological specimens from the oral mucosa of patients with oral submucous fibrosis. Furthermore, the same author (Pindborg et al., 1964) reports an incidence of leukoplakia in 28 per cent of patients affected with submucous fibrosis, which is nine fold higher than the control population. References Binnie, W. H. & Cawson, R. A. (1972). A new ultrastructural British
Journal of Dermatology,
finding in oral submucous
fibrosis.
86, 286.
Hammer, J. E. III, Mehta, F. S., Pindborg, J. J. & Daftary, D. K. (1971). Altered staining reaction of connective tissues in 53 submucous fibrosis patients. Journal of Dental Research, 50, 388. Joshi, S. G. (1953). Submucous fibrosis of the palate and pillars. Indian Journalof Otolaryngology, 4,1. Kennedy, T. F. & MacDonald, D. G. (1968). Oral submucous fibrosis. A case report. British Dental Journal, 124, 121. Lal, D. (1953). Diffuse oral submucous fibrosis. Journal of All-Zndia Dental Association, 26, 1. Pindborg, J. J., Chawla, T. N., Srivastava, A. N., Gupta, D. & Mehrotra, M. L. (1964). Clinical aspects of oral submucous fibrosis. Acta Odontologieal Scandinavica, 22, 679. Pindborg, J. J. & Sirsat, S. M. (1966). Oral submucous fibrosis. Oral Surgery, Oral Medicine and Oral Pathology,
22, 764.
ORAL
SUBMUCOUS
FIBROSIS
IN
Pindborg, J. J. (1972). Is submucous fibrosis a precancerous Dental Journal, 22, 474. Pindborg, J. J. (1980). Oral Cancer and Precancer.
A
GREEK
FEMALE
201
condition in the oral cavity? Znternntional
Bristol: Wright. Rao, A. B. N. (1962). Idiopathic palatal fibrosis. British Journal of Surgery, 50, 23. Schwartz, J. (1952). Atrophic idiopathica (tropica) mucosaeoris. Demonstrated at the 11th International Dental Congress, London. Simpson, W. (1969). Submucous fibrosis. British Journal of Oral Surgery, 6, 196.