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Mikulicz's disease involving multiple salivary glands
Report of a Case James E. Lancaster, Lieutenant Kenneth W. Hughes, Lieutenant
Colonel, DC, USA,” and Colonel, DC, TJSA”’
disease was first described by John von Mikulicz in 1888 as a benign enlargement of the lacrimal and the salivary g1ands.l Since the time of Mikulicz’s original description, etiological factors have never been conelusively identified.* The most consistent prominent clinical feature of the disease is the painless enlargement of lacrimal and salivary glands.3 Histologic features of involved glandular tissues are characterized by infiltration of glandular structures with lymphocytic elements’ in either a nodular or a diffuse microscopic pattern. Because of the benign nature of the lesion, it, has been recommended that treatment bc conservative.’
M
CASE
IKULICZ’S
REPORT
Chief Complaint.--A G-year-old Caucasian male soldier presented to the Fort Dix Dental Service on Sept. 29, 1960, complaining of a painless swelling in the left post,erior portion of the hard palate which interfered with the insertion of his partial denture. Ilktory of Present Illness.-Approximately one year prior to admission, the patient first noted the appearance of a small, moderately firm, painless mass involving the mucosa on the left posterior portion of the hard palate; he subsequently observed its gradual increase in size. The lesion, which protruded above t,he level of the surrounding soft tissues, caused no pain or discomfort but created increasing interference in the retention of a partial denture. There was no history of ulceration, bleeding, rupture, or discharge associated with the lesion. Past Medical History.-History elicited from the patient indicated that he had noticed a gradual enlargement of tissues in the area of both parotid glands and the right submaxillary gland during the past 6 years. The symptoms were associated with minor complaints 2 years prior to this admission of conjunctivitis and ‘ ‘ dryness of the eyes. ” Approximately the, pa,tient had been seen at another hospital and had been released after evaluation, which included sialographic studies. During the same period the patient had suffered intermit,tent episodes of acute pain and smelling about the salivary and lacrimal glands; these episodes were usually associated with upper respiratory infections. The pa,tient believed that he had noted a gradual enlargement of the right submaxillary gland during the past several months. This material has been reviewed by the OWce of the Surgeon General. Department of the Army, and there is no objection to its presentation and/or publication. This review does not imply any indorsement of the opinions advanced or any recommendations of such products as may be named. *Chief, Oral Diagnosis and Roentgenology, Dental Service, Fort Hood, Texas. **Chief, Oral Surgery, Dental Service, Fort Dix, N. J.
1266
MIKULlCZ’S
Volume 16 Number IO
DISEASE
1267
Personal History.-The patient was employed as a musician in the brass wind instrument (French horn) section of a band. The remainder of the personal, family, and SOCd histories was not considered pertinent. Physical Esamination.-Clinical examination upon admission revealed a thin, apprewas essentially negative except for hensive man in no apparent acute distress. Examination was present. findings about the face and oral cavity. A moderate degree of conjunctivitis
Fig.
l.-Swelling
Fig.
Z.-Mass
of
located
salivary
in hard
glands.
palate.
There was an irregular nodular enlargement of each parotid gland, which was nontender to palpation and evidenced a firm, doughy consistency. The enlargement of the left parotid gland extended well below the angle of the mandible, and there was a similar nontender nodular enlargement of the right submaxillary gland which contained one well-defined nodule measuring approximately 1 cm. in diameter. Examination of the oral cavity was essentially negative except for the presence of a slightly compressible nontender mass, approximately
I.5 cm. in diameter, protruding from the left posterior aspect of’ t,he hard palate and a somewhat indiscrete soft mass which was palpabh deep within thtl suhstanw of the right aspect of the soft palate. Then mass on the hart? palat appeawd to Ike slightly excoriated where it was contacttd 1)~ the, partial tl(~nture.
Fig.
Fig. 4.-Histologic
3..--Gross
appearance
appearance
of pal&al
of submaxillary
.r&nd
mass tlemonstrating
specimen.
lymphocytic
infiltration
Laboratory Studies.-The white blood count was 5,100 (neutrophils, 42 ; lymphocytes, 44; monocytes, 0; eosinophils, 14). Hemoglobin Iv-as 14.0. The bleeding time was 1 minute 10 seconds, and the coagulation time was 13 minutes. Urinalysis was within normal limits. Roentgenographic Stzldirs.-Routine chest films were negative. Intraoral occlusal films revealed a small amount of roentgenopaque material in the area of the right, parotid gland. Subsequent sialography of the parotid and submaxillary glands revealed marked sialectasis and delayed retention of contrast media.
Volllme
16
Number lo
MIKULZCZ
‘S DISEASE
1269
Treatment.-During the month of September, 1960, the mass in the hard palate and the entire right submaxillary gland were removed on separate occasions under local anesthesia. The procedures were uncomplicated, and the patient tolerated them well. The postoperative course after each procedure was completely uneventful, with normal repair occurring at the operative sites. Grossly, the cut specimens appeared to be well encapsulated; they were w,hite in appearance and relatively avascular. Both specimens were submitted for histologic examination. Pathologist’s Report.Palatal lesion: Examination revealed the tissue to be lined on one surface by stratified squamous epithelium. The underlying tissue was densely infiltrated by lymphoid tissue. Widely separated ductal elements showed proliferation of the lining cells. Irregularly shaped islands of myoepithelial cells were present within the lymphoid infiltrate. Diagnosis: Lymphoid infiltrate compatible with Mikulicz ‘s disease. Right &mazilZaary gland: The glandular elements were almost totally obliterated by a rich lymphocyte infiltration with focal germinal centers. The few remaining ductal elements were occluded by squamous metaplasia and lymphocyte infiltration. The epithelium of these ductal elements was quite orderly. No acinar tissue remained. Dia,qmosis: Submaxillary gland, so-called Mikulicz ‘s disease. FoZZowt-up Report.-On March 21, 1961, the patient returned with complaints of acute, swelling and discomfort about both parotid glands. Examination revealed a purulent discharge exuding from Stensen’s ducts bilaterally. Bacterial examination of the purulent material was positive for DipZococ~us pnevmoniae and a diagnosis of bacterial parotitis was made. The patient was placed on antibiotic therapy consisting of 1 Gm. of tetracycline daily in divided doses for 5 days. Forty-eight hours following the initiation of therapy the pain, swelling, and discharge had subsided. There was no further recurrence of the parotitis during the subsequent 2 months of observation. DISCUSSION
This case is interesting because of the involvement of palatal salivary glands in Xlikulicz’s disease. There have been very few reports in which palatal glands were affected. The episode of parotitis in this case could certainly be expected because of decreased function and subsequent stasis within the glands. This patient will be observed further, since there is little doubt that all symptomatic glands’are involved with the same disease process. SUMMARY
1. A case of multiglandular Mikulicz’s disease has been presented. ‘1. Parotitis could be expected as a complication of this disease because of decreased function and resulting stasis. 3. Conservative treatment will be followed, since a definite diagnosis has been made. REFERENCES 1. Mikulicz, J. von: Concerning a Peculiar Disease of Lacrymal and Salivary Glands, M. Classics 2: 165-186, 1937. 2. Cheraskin, E., and Langley, L. L.: Dynamics of Oral Diagnosis, Chicago, 1956, Year Book Publishers, Inc. 3. Thoma, K. H., and Goldman, H. M.: Oral Pathology, ed. 5, St. Louis, 1960, The C. V. Mosby Company. 4. Shafer, W. G., Hine, M. K., and Levy, B. M.: A Textbook of Oral Pathology, Philadelphia, 1959, W. B. Saunders Company. 3. Bernier, J. L., and Bhaskar, S. N.: Mikuliez’s Disease, ORAL SURG., ORAL MED. & ORAL PATH. 13: 1387-1399, 1960.
Colonel, DC, USA,” and Colonel, DC, TJSA”’
disease was first described by John von Mikulicz in 1888 as a benign enlargement of the lacrimal and the salivary g1ands.l Since the time of Mikulicz’s original description, etiological factors have never been conelusively identified.* The most consistent prominent clinical feature of the disease is the painless enlargement of lacrimal and salivary glands.3 Histologic features of involved glandular tissues are characterized by infiltration of glandular structures with lymphocytic elements’ in either a nodular or a diffuse microscopic pattern. Because of the benign nature of the lesion, it, has been recommended that treatment bc conservative.’
M
CASE
IKULICZ’S
REPORT
Chief Complaint.--A G-year-old Caucasian male soldier presented to the Fort Dix Dental Service on Sept. 29, 1960, complaining of a painless swelling in the left post,erior portion of the hard palate which interfered with the insertion of his partial denture. Ilktory of Present Illness.-Approximately one year prior to admission, the patient first noted the appearance of a small, moderately firm, painless mass involving the mucosa on the left posterior portion of the hard palate; he subsequently observed its gradual increase in size. The lesion, which protruded above t,he level of the surrounding soft tissues, caused no pain or discomfort but created increasing interference in the retention of a partial denture. There was no history of ulceration, bleeding, rupture, or discharge associated with the lesion. Past Medical History.-History elicited from the patient indicated that he had noticed a gradual enlargement of tissues in the area of both parotid glands and the right submaxillary gland during the past 6 years. The symptoms were associated with minor complaints 2 years prior to this admission of conjunctivitis and ‘ ‘ dryness of the eyes. ” Approximately the, pa,tient had been seen at another hospital and had been released after evaluation, which included sialographic studies. During the same period the patient had suffered intermit,tent episodes of acute pain and smelling about the salivary and lacrimal glands; these episodes were usually associated with upper respiratory infections. The pa,tient believed that he had noted a gradual enlargement of the right submaxillary gland during the past several months. This material has been reviewed by the OWce of the Surgeon General. Department of the Army, and there is no objection to its presentation and/or publication. This review does not imply any indorsement of the opinions advanced or any recommendations of such products as may be named. *Chief, Oral Diagnosis and Roentgenology, Dental Service, Fort Hood, Texas. **Chief, Oral Surgery, Dental Service, Fort Dix, N. J.
1266
MIKULlCZ’S
Volume 16 Number IO
DISEASE
1267
Personal History.-The patient was employed as a musician in the brass wind instrument (French horn) section of a band. The remainder of the personal, family, and SOCd histories was not considered pertinent. Physical Esamination.-Clinical examination upon admission revealed a thin, apprewas essentially negative except for hensive man in no apparent acute distress. Examination was present. findings about the face and oral cavity. A moderate degree of conjunctivitis
Fig.
l.-Swelling
Fig.
Z.-Mass
of
located
salivary
in hard
glands.
palate.
There was an irregular nodular enlargement of each parotid gland, which was nontender to palpation and evidenced a firm, doughy consistency. The enlargement of the left parotid gland extended well below the angle of the mandible, and there was a similar nontender nodular enlargement of the right submaxillary gland which contained one well-defined nodule measuring approximately 1 cm. in diameter. Examination of the oral cavity was essentially negative except for the presence of a slightly compressible nontender mass, approximately
I.5 cm. in diameter, protruding from the left posterior aspect of’ t,he hard palate and a somewhat indiscrete soft mass which was palpabh deep within thtl suhstanw of the right aspect of the soft palate. Then mass on the hart? palat appeawd to Ike slightly excoriated where it was contacttd 1)~ the, partial tl(~nture.
Fig.
Fig. 4.-Histologic
3..--Gross
appearance
appearance
of pal&al
of submaxillary
.r&nd
mass tlemonstrating
specimen.
lymphocytic
infiltration
Laboratory Studies.-The white blood count was 5,100 (neutrophils, 42 ; lymphocytes, 44; monocytes, 0; eosinophils, 14). Hemoglobin Iv-as 14.0. The bleeding time was 1 minute 10 seconds, and the coagulation time was 13 minutes. Urinalysis was within normal limits. Roentgenographic Stzldirs.-Routine chest films were negative. Intraoral occlusal films revealed a small amount of roentgenopaque material in the area of the right, parotid gland. Subsequent sialography of the parotid and submaxillary glands revealed marked sialectasis and delayed retention of contrast media.
Volllme
16
Number lo
MIKULZCZ
‘S DISEASE
1269
Treatment.-During the month of September, 1960, the mass in the hard palate and the entire right submaxillary gland were removed on separate occasions under local anesthesia. The procedures were uncomplicated, and the patient tolerated them well. The postoperative course after each procedure was completely uneventful, with normal repair occurring at the operative sites. Grossly, the cut specimens appeared to be well encapsulated; they were w,hite in appearance and relatively avascular. Both specimens were submitted for histologic examination. Pathologist’s Report.Palatal lesion: Examination revealed the tissue to be lined on one surface by stratified squamous epithelium. The underlying tissue was densely infiltrated by lymphoid tissue. Widely separated ductal elements showed proliferation of the lining cells. Irregularly shaped islands of myoepithelial cells were present within the lymphoid infiltrate. Diagnosis: Lymphoid infiltrate compatible with Mikulicz ‘s disease. Right &mazilZaary gland: The glandular elements were almost totally obliterated by a rich lymphocyte infiltration with focal germinal centers. The few remaining ductal elements were occluded by squamous metaplasia and lymphocyte infiltration. The epithelium of these ductal elements was quite orderly. No acinar tissue remained. Dia,qmosis: Submaxillary gland, so-called Mikulicz ‘s disease. FoZZowt-up Report.-On March 21, 1961, the patient returned with complaints of acute, swelling and discomfort about both parotid glands. Examination revealed a purulent discharge exuding from Stensen’s ducts bilaterally. Bacterial examination of the purulent material was positive for DipZococ~us pnevmoniae and a diagnosis of bacterial parotitis was made. The patient was placed on antibiotic therapy consisting of 1 Gm. of tetracycline daily in divided doses for 5 days. Forty-eight hours following the initiation of therapy the pain, swelling, and discharge had subsided. There was no further recurrence of the parotitis during the subsequent 2 months of observation. DISCUSSION
This case is interesting because of the involvement of palatal salivary glands in Xlikulicz’s disease. There have been very few reports in which palatal glands were affected. The episode of parotitis in this case could certainly be expected because of decreased function and subsequent stasis within the glands. This patient will be observed further, since there is little doubt that all symptomatic glands’are involved with the same disease process. SUMMARY
1. A case of multiglandular Mikulicz’s disease has been presented. ‘1. Parotitis could be expected as a complication of this disease because of decreased function and resulting stasis. 3. Conservative treatment will be followed, since a definite diagnosis has been made. REFERENCES 1. Mikulicz, J. von: Concerning a Peculiar Disease of Lacrymal and Salivary Glands, M. Classics 2: 165-186, 1937. 2. Cheraskin, E., and Langley, L. L.: Dynamics of Oral Diagnosis, Chicago, 1956, Year Book Publishers, Inc. 3. Thoma, K. H., and Goldman, H. M.: Oral Pathology, ed. 5, St. Louis, 1960, The C. V. Mosby Company. 4. Shafer, W. G., Hine, M. K., and Levy, B. M.: A Textbook of Oral Pathology, Philadelphia, 1959, W. B. Saunders Company. 3. Bernier, J. L., and Bhaskar, S. N.: Mikuliez’s Disease, ORAL SURG., ORAL MED. & ORAL PATH. 13: 1387-1399, 1960.