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Oral sarcoidosis with tongue involvement
Oral sarcoidosis with tongue involvement A. Serrat Soto, M D , a P. L o b o Valentin, M D , b L. M. R e d o n d o Gonz~ilez, M D , PhD, c C. Sanz Santa Cruz, M D , PhD, d and A. V e r r i e r Hernfindez, M D , PhD, e Valladolid, Spain "DEL RIO HORTEGA" HOSPITAL, REGIONAL DEPARTMENT OF ORAL AND MAXILLOFACIAL SURGERY A case of oral sarcoidosis involving the tongue and buccal region is reported in a 56-year-old woman. Intraoral presentations of sarcoidosis are uncommon, and sarcoidosis of the tongue is particularly rare. In this case the tongue lesion was asymptomatic, an unusual clinical behavior. In this article, we review the clinical manifestations, diagnostic procedures, differential diagnosis and treatments of oral sarcoidosis. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;83:668-71)
S a r c o i d o s i s is a m u l t i s y s t e m g r a n u l o m a t o u s d i s e a s e o f u n k n o w n cause that m a y affect any organ. It m a n ifests m o s t frequently with bilateral hilar l y m p h a d e nopathy, p u l m o n a r y infiltration, and skin or e y e lesions. H i s t o l o g i c a l l y , the h a l l m a r k o f this d i s e a s e is a noncaseating, e p i t h e l i o i d - c e l l granuloma. T h e onset o f sarcoidosis is m o s t c o m m o n l y seen b e t w e e n 20 and 40 years o f age. In the U n i t e d States the m a j o r i t y o f patients are b l a c k with a b l a c k - t o - w h i t e patient ratio o f 10.17:1. S a r c o i d o s i s is slightly m o r e c o m m o n in w o m e n than in m e n 1. T h e d i a g n o s i s is e s t a b l i s h e d w h e n clinical and r a d i o g r a p h i c findings are s u p p o r t e d b y histologic e v i d e n c e o f n o n c a s e a t i n g e p i t h e l i o i d cell g r a n u l o m a s . 1 Oral presentations o f sarcoidosis, unrelated to l y m p h n o d e or salivary g l a n d disease, h a v e b e e n rep o r t e d infrequently. R e p o r t e d sites o f oral i n v o l v e m e n t h a v e i n c l u d e d the hard and soft palate, 2 lips, 2, 3 tongue, 4-7 g i n g i v a ? and t o n s i l ? but the d i s e a s e m a y affect any site o f the mouth.1 Oral lesions h a v e typi c a l l y b e e n d e s c r i b e d as nontender, w e l l - c i r c u m scribed, b r o w n i s h red or v i o l a c e o u s nodules or p a p ules that o c c a s i o n a l l y s h o w superficial ulceration. 2 T h e p u r p o s e o f this article is to r e p o r t a case o f oral sarcoidosis i n v o l v i n g the tongue and b u c c a l region. S a r c o i d o s i s o f the tongue is p a r t i c u l a r l y rare. A l though oral lesions t y p i c a l l y h a v e b e e n d e s c r i b e d as nontender, s a r c o i d o s i s o f the tongue has u s u a l l y b e e n
aResident. bResident. ~Associate Professor. dChief Section, Department of Pathology. eChief Department. Received for publication April 30, 1996; returned for revision June 5, 1996; accepted for publication July 22, 1996. Copyright © 1997 by Mosby-Year Book, Inc. 1079-2104/97/$5.00 + 0 7/13/78911 668
r e p o r t e d with s y m p t o m a t i c manifestations. In our p a tient the tongue lesions w e r e a s y m p t o m a t i c .
CASE REPORT A 56-year-old white woman was referred to our service for evaluation and treatment of a lesion on the ventral surface of the tongue. Her medical history included a diagnosis of systemic sarcoidosis with pulmonary involvement of several years' duration. Intraoral examination showed a 2 x 3 cm erosive, papular lesion on the right ventral surface of the tongue (Fig. 1). The lesion had been present for 5 months and was slowly enlarging. Additionally, she had a 1.5 cm submucosal nodule in the left commissure of the mouth that had become apparent about 19 months before the lesion on the tongue (Fig. 2). Both lesions were asymptomatic. The patient had no difficulty in eating or talking, and the motility of the tongue was nor reduced. No cervical lymph nodes were palpable, and the salivary glands were not enlarged. The remainder of the head and neck examination was normal. Under local anesthesia, an incisional biopsy was taken from the lesion of the tongue. Histologic examination revealed numerous epithelioid cell granulomas (Fig. 3), with a variable rim of histiocytes and lymphoid cells surrounding the granuloma, and Langerhans' giant cells (Fig. 4). Central necroses were not found, nor was there microscopic evidence of acid-fast bacilli, fungi, or birefringent foreign material. We also performed a fine-needle aspiration biopsy from the buccal nodule. Microscopic examination of the specimen showed the presence of epithelioid cells mixed with multinucleate giant cells in a granulomatous pattern. The patient's medical history, the characteristic histologic finding, and a negative special stain for acid-fast bacilli and fungi confirmed the diagnosis of oral sarcoidosis. The patient required systemic corticosteroids to control other aspects of her disease; drug therapy was initiated with prednisone, 40 mg daily. The prednisone dose was reduced weekly. At follow-up examinations, the patient showed a marked reduction in the intraoral lesions.
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY
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Volume 83, Number 6
Fig. 1. Erosive papular lesion on the right ventral surface of the tongue (arrows).
DISCUSSION The most common cervicofacial manifestation of sarcoidosis, except ocular and lacrimal gland involvement, appears to be asymptomatic swelling of the parotid glands or cervical nodes. 1° Cutaneous sarcoidosis of the facial skin is generally recognizable as a nodule, maculopapules, plaques, or lupus pernio (violaceous infiltrated nodules and plaques that occur more or less symmetrically on the nose, cheeks, ears, etc.).1, 11 Sarcoidosis lesions of the facial bones have been reported. 2, 11, 12 Osteoporosis, cortical thinning, a well-defined cyst, and extensive bone destruction have been described. The facial nerve is frequently affected, with or without Heerfordt's disease (uveitis, parotid gland enlargement, fever, and facial nerve palsy). 1 Sarcoid granulomas may be found on the nasal mucosa, 1 larynx] and paranasal sinuses, u Intraoral presentation of sarcoidosis is uncommon and, in most cases, sarcoidosis has been diagnosed before the oral manifestation became apparent. 4 One study reported a 38 to 58 percent presence of noncaseating granulomas in biopsies of normal-appearing oral mucosa of patients with known sarcoidosis. 13 Oral involvement generally appears in patients with chronic multisystem sarcoidosis and very seldom occurs in the acute stageJ 4 The tongue is rarely involved in sarcoidosis; to our knowledge, only been a few cases have been reported. 4-7 Oral lesions have typically been described as nontender, but occasionally may be symptomatic. Sar-
Fig. 2. Nodular lesion in the buccal submucosa (arrows).
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ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY June 1997
Fig. 3. Multiple epithelioid cell granulomas without central necrosis. (Hematoxylin-eosin stain; original magnification xl00.)
Fig. 4. A multinucleate giant cell (Langerhans-type) in an epithelioid stroma (arrow). (Hematoxylin-eosin stain; original magnification xl00.)
coidosis of the tongue may be asymptomatic (our patient) or mildly symptomatic 4 with minimal discomfort when eating or drinking. Other case reports have described lesions of the tongue that caused severe discomfort when eating or drinking, leading to weight
loss, dysarthria, deterioration in tongue mobility or, difficulty in swallowing. 5-7 The diagnosis is established when clinical findings are supported by histologic evidence of typical granulomas so as to exclude other diseases that produce
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Volume 83, Number 6
granulomas.l The histologic presentation of the true sarcoidosis cannot always be distinguished from that of sarcoidal granulomas from other causes. Bacterial, fungal, and viral infections should be borne in mind when establishing a differential diagnosis of oral sarcoidosis. 1 Tuberculosis, syphilis, histoplasmosis, actinomycosis, and other infections may produce a sarcoidal type of tissue response or granulomas mimicking sarcoidosis. Differential diagnostic possibilities include foreign body granulomas and orofacial granulomatosis, such as oral Crohn's disease, granulomatous cheilitis, and Melkersson's syndrome. Carcinoma of the oral cavity should always be considered in the differential diagnosis of oral sarcoidosis. The chance of spontaneous remission favors a conservative approach to systemic therapy, which is usually carried out with corticosteroids. At any time the disease pattern may change, but an expectant policy is often better if the course is not progressive and if vital structures are not involved. 1 In oral sarcoidosis any decision about the use of systemic therapy must take into account the seriousness of the accompanying internal involvement and the symptoms of oral lesions. Most oral sarcoidosis is treated with corticosteroids,2, 3, 5, 7, 11 but mild asymptomatic cases may not require systemic therapy. 4 Surgical excision has been used to remove oral lesions 3 or fibrotic bands. 7 REFERENCES 1. Crystal RG. Sarcoidosis. In: Isselbacher KJ, Braunwald E, Wilson JD, et al, eds: Harrison. Principios de Medicina
Soto et al.
2.
3. 4. 5.
6. 7.
8. 9. 10. 11.
12.
13.
14.
671
Interna. Madrid: McGraw-Hill Interamericana, 1994: 1932-8. Hildebrand J, Plezia RA, Rao SB. Sarcoidosis: report of two cases with oral involvement. Oral Surg Oral Med Oral Pathol 1990;69:217-22. Steinberg MJ, Mueller DP. Treating oral sarcoidosis. J A m Dent Assoc 1994;125:76-9. Mendelsohn SS, Field EA, Woolgar J. Sarcoidosis of the tongue. Clin Exp Dermatol 1992;17:47-8. Pila P6rez R, Pila Pel~tez M, Pila Pel~ez R, et al. Sarcoidosis of the tongue: report of a case. Rev Esp E n f Diget 1991;80: 201-3. Tillman HH, Taylor RG, Carchidi JE. Sarcoidosis of the tongue. Oral Surg Oral Meal Oral Pathol 1966;21:190-~5. Macleod RI, Snow MH, Hawkesford JE. Sarcoidosis of the tongue: a case report. Br J Oral Maxillofac Surg 1985; 23:243-6. Hayter JP, Robertson JM. Sarcoidosis presenting as gingivitis. Br Med J 1988;296:1504-5. Erwin SA. Unsuspected sarcoidosis of the tonsil. Otolaryngol Head Neck Surg 1989;100:245-7. Gold RS, Sager E. Oral sarcoidosis: review of the literature. J Oral Surg 1976;34:237-44. Ktesper B, Schmelzle R, Donath K. Cutaneous manifestation of sarcoidosis (Boeck) with severe osseous destruction of the midface. J Craniomaxillofac Surg 1994;22:163-6. Rubin MM, Sanfilippo RJ, Pliskin A. Maxillary alveolar bone loss in a patient with sarcoidosis. J Oral Maxillofac Surg 1991;49:1351-3. Van Maarsseveen A C M TH, Van der Waal Y, Stam J, et al. Oral involvement in sarcoidosis. Int J Oral Surg Oral Med Oral Pathol 1982;11:21-9. Wilson R, Lunal V, Sweatman M, et al. Upper respiratory tract involvement in sarcoidosis and its management. Eur Respir J 1988; 1:269-72.
Reprint requests: A. Serrat Soto, MD C! Las Mercedes, 7, 1° C. 47006 Valladolid, Spain
S a r c o i d o s i s is a m u l t i s y s t e m g r a n u l o m a t o u s d i s e a s e o f u n k n o w n cause that m a y affect any organ. It m a n ifests m o s t frequently with bilateral hilar l y m p h a d e nopathy, p u l m o n a r y infiltration, and skin or e y e lesions. H i s t o l o g i c a l l y , the h a l l m a r k o f this d i s e a s e is a noncaseating, e p i t h e l i o i d - c e l l granuloma. T h e onset o f sarcoidosis is m o s t c o m m o n l y seen b e t w e e n 20 and 40 years o f age. In the U n i t e d States the m a j o r i t y o f patients are b l a c k with a b l a c k - t o - w h i t e patient ratio o f 10.17:1. S a r c o i d o s i s is slightly m o r e c o m m o n in w o m e n than in m e n 1. T h e d i a g n o s i s is e s t a b l i s h e d w h e n clinical and r a d i o g r a p h i c findings are s u p p o r t e d b y histologic e v i d e n c e o f n o n c a s e a t i n g e p i t h e l i o i d cell g r a n u l o m a s . 1 Oral presentations o f sarcoidosis, unrelated to l y m p h n o d e or salivary g l a n d disease, h a v e b e e n rep o r t e d infrequently. R e p o r t e d sites o f oral i n v o l v e m e n t h a v e i n c l u d e d the hard and soft palate, 2 lips, 2, 3 tongue, 4-7 g i n g i v a ? and t o n s i l ? but the d i s e a s e m a y affect any site o f the mouth.1 Oral lesions h a v e typi c a l l y b e e n d e s c r i b e d as nontender, w e l l - c i r c u m scribed, b r o w n i s h red or v i o l a c e o u s nodules or p a p ules that o c c a s i o n a l l y s h o w superficial ulceration. 2 T h e p u r p o s e o f this article is to r e p o r t a case o f oral sarcoidosis i n v o l v i n g the tongue and b u c c a l region. S a r c o i d o s i s o f the tongue is p a r t i c u l a r l y rare. A l though oral lesions t y p i c a l l y h a v e b e e n d e s c r i b e d as nontender, s a r c o i d o s i s o f the tongue has u s u a l l y b e e n
aResident. bResident. ~Associate Professor. dChief Section, Department of Pathology. eChief Department. Received for publication April 30, 1996; returned for revision June 5, 1996; accepted for publication July 22, 1996. Copyright © 1997 by Mosby-Year Book, Inc. 1079-2104/97/$5.00 + 0 7/13/78911 668
r e p o r t e d with s y m p t o m a t i c manifestations. In our p a tient the tongue lesions w e r e a s y m p t o m a t i c .
CASE REPORT A 56-year-old white woman was referred to our service for evaluation and treatment of a lesion on the ventral surface of the tongue. Her medical history included a diagnosis of systemic sarcoidosis with pulmonary involvement of several years' duration. Intraoral examination showed a 2 x 3 cm erosive, papular lesion on the right ventral surface of the tongue (Fig. 1). The lesion had been present for 5 months and was slowly enlarging. Additionally, she had a 1.5 cm submucosal nodule in the left commissure of the mouth that had become apparent about 19 months before the lesion on the tongue (Fig. 2). Both lesions were asymptomatic. The patient had no difficulty in eating or talking, and the motility of the tongue was nor reduced. No cervical lymph nodes were palpable, and the salivary glands were not enlarged. The remainder of the head and neck examination was normal. Under local anesthesia, an incisional biopsy was taken from the lesion of the tongue. Histologic examination revealed numerous epithelioid cell granulomas (Fig. 3), with a variable rim of histiocytes and lymphoid cells surrounding the granuloma, and Langerhans' giant cells (Fig. 4). Central necroses were not found, nor was there microscopic evidence of acid-fast bacilli, fungi, or birefringent foreign material. We also performed a fine-needle aspiration biopsy from the buccal nodule. Microscopic examination of the specimen showed the presence of epithelioid cells mixed with multinucleate giant cells in a granulomatous pattern. The patient's medical history, the characteristic histologic finding, and a negative special stain for acid-fast bacilli and fungi confirmed the diagnosis of oral sarcoidosis. The patient required systemic corticosteroids to control other aspects of her disease; drug therapy was initiated with prednisone, 40 mg daily. The prednisone dose was reduced weekly. At follow-up examinations, the patient showed a marked reduction in the intraoral lesions.
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY
Soto et al.
669
Volume 83, Number 6
Fig. 1. Erosive papular lesion on the right ventral surface of the tongue (arrows).
DISCUSSION The most common cervicofacial manifestation of sarcoidosis, except ocular and lacrimal gland involvement, appears to be asymptomatic swelling of the parotid glands or cervical nodes. 1° Cutaneous sarcoidosis of the facial skin is generally recognizable as a nodule, maculopapules, plaques, or lupus pernio (violaceous infiltrated nodules and plaques that occur more or less symmetrically on the nose, cheeks, ears, etc.).1, 11 Sarcoidosis lesions of the facial bones have been reported. 2, 11, 12 Osteoporosis, cortical thinning, a well-defined cyst, and extensive bone destruction have been described. The facial nerve is frequently affected, with or without Heerfordt's disease (uveitis, parotid gland enlargement, fever, and facial nerve palsy). 1 Sarcoid granulomas may be found on the nasal mucosa, 1 larynx] and paranasal sinuses, u Intraoral presentation of sarcoidosis is uncommon and, in most cases, sarcoidosis has been diagnosed before the oral manifestation became apparent. 4 One study reported a 38 to 58 percent presence of noncaseating granulomas in biopsies of normal-appearing oral mucosa of patients with known sarcoidosis. 13 Oral involvement generally appears in patients with chronic multisystem sarcoidosis and very seldom occurs in the acute stageJ 4 The tongue is rarely involved in sarcoidosis; to our knowledge, only been a few cases have been reported. 4-7 Oral lesions have typically been described as nontender, but occasionally may be symptomatic. Sar-
Fig. 2. Nodular lesion in the buccal submucosa (arrows).
670
Soto et aL
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY June 1997
Fig. 3. Multiple epithelioid cell granulomas without central necrosis. (Hematoxylin-eosin stain; original magnification xl00.)
Fig. 4. A multinucleate giant cell (Langerhans-type) in an epithelioid stroma (arrow). (Hematoxylin-eosin stain; original magnification xl00.)
coidosis of the tongue may be asymptomatic (our patient) or mildly symptomatic 4 with minimal discomfort when eating or drinking. Other case reports have described lesions of the tongue that caused severe discomfort when eating or drinking, leading to weight
loss, dysarthria, deterioration in tongue mobility or, difficulty in swallowing. 5-7 The diagnosis is established when clinical findings are supported by histologic evidence of typical granulomas so as to exclude other diseases that produce
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Volume 83, Number 6
granulomas.l The histologic presentation of the true sarcoidosis cannot always be distinguished from that of sarcoidal granulomas from other causes. Bacterial, fungal, and viral infections should be borne in mind when establishing a differential diagnosis of oral sarcoidosis. 1 Tuberculosis, syphilis, histoplasmosis, actinomycosis, and other infections may produce a sarcoidal type of tissue response or granulomas mimicking sarcoidosis. Differential diagnostic possibilities include foreign body granulomas and orofacial granulomatosis, such as oral Crohn's disease, granulomatous cheilitis, and Melkersson's syndrome. Carcinoma of the oral cavity should always be considered in the differential diagnosis of oral sarcoidosis. The chance of spontaneous remission favors a conservative approach to systemic therapy, which is usually carried out with corticosteroids. At any time the disease pattern may change, but an expectant policy is often better if the course is not progressive and if vital structures are not involved. 1 In oral sarcoidosis any decision about the use of systemic therapy must take into account the seriousness of the accompanying internal involvement and the symptoms of oral lesions. Most oral sarcoidosis is treated with corticosteroids,2, 3, 5, 7, 11 but mild asymptomatic cases may not require systemic therapy. 4 Surgical excision has been used to remove oral lesions 3 or fibrotic bands. 7 REFERENCES 1. Crystal RG. Sarcoidosis. In: Isselbacher KJ, Braunwald E, Wilson JD, et al, eds: Harrison. Principios de Medicina
Soto et al.
2.
3. 4. 5.
6. 7.
8. 9. 10. 11.
12.
13.
14.
671
Interna. Madrid: McGraw-Hill Interamericana, 1994: 1932-8. Hildebrand J, Plezia RA, Rao SB. Sarcoidosis: report of two cases with oral involvement. Oral Surg Oral Med Oral Pathol 1990;69:217-22. Steinberg MJ, Mueller DP. Treating oral sarcoidosis. J A m Dent Assoc 1994;125:76-9. Mendelsohn SS, Field EA, Woolgar J. Sarcoidosis of the tongue. Clin Exp Dermatol 1992;17:47-8. Pila P6rez R, Pila Pel~tez M, Pila Pel~ez R, et al. Sarcoidosis of the tongue: report of a case. Rev Esp E n f Diget 1991;80: 201-3. Tillman HH, Taylor RG, Carchidi JE. Sarcoidosis of the tongue. Oral Surg Oral Meal Oral Pathol 1966;21:190-~5. Macleod RI, Snow MH, Hawkesford JE. Sarcoidosis of the tongue: a case report. Br J Oral Maxillofac Surg 1985; 23:243-6. Hayter JP, Robertson JM. Sarcoidosis presenting as gingivitis. Br Med J 1988;296:1504-5. Erwin SA. Unsuspected sarcoidosis of the tonsil. Otolaryngol Head Neck Surg 1989;100:245-7. Gold RS, Sager E. Oral sarcoidosis: review of the literature. J Oral Surg 1976;34:237-44. Ktesper B, Schmelzle R, Donath K. Cutaneous manifestation of sarcoidosis (Boeck) with severe osseous destruction of the midface. J Craniomaxillofac Surg 1994;22:163-6. Rubin MM, Sanfilippo RJ, Pliskin A. Maxillary alveolar bone loss in a patient with sarcoidosis. J Oral Maxillofac Surg 1991;49:1351-3. Van Maarsseveen A C M TH, Van der Waal Y, Stam J, et al. Oral involvement in sarcoidosis. Int J Oral Surg Oral Med Oral Pathol 1982;11:21-9. Wilson R, Lunal V, Sweatman M, et al. Upper respiratory tract involvement in sarcoidosis and its management. Eur Respir J 1988; 1:269-72.
Reprint requests: A. Serrat Soto, MD C! Las Mercedes, 7, 1° C. 47006 Valladolid, Spain