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ARTICLES
Year : 1975  |  Volume : 23  |  Issue : 2  |  Page : 27-28

Mikulciz's disease


1 Dept of Ophthalmology, Jawaharlal Nehru Medical College, Belgaum, India
2 Dept of Pathology, Jawaharlal Nehru Medical College, Belgaum, India

Correspondence Address:
S J Naglotimath
Dept of Pathology, Jawaharlal Nehru Medical College, Belgaum
India
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Source of Support: None, Conflict of Interest: None


PMID: 1236451

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How to cite this article:
Veerappa T S, Naglotimath S J. Mikulciz's disease. Indian J Ophthalmol 1975;23:27-8

How to cite this URL:
Veerappa T S, Naglotimath S J. Mikulciz's disease. Indian J Ophthalmol [serial online] 1975 [cited 2020 Mar 29];23:27-8. Available from: http://www.ijo.in/text.asp?1975/23/2/27/31321

Mikulicz described bilateral symmetrical enlargement of the lacrimal and salivary glands as an independent affection in 1885. Since then the disease marked by enlargement of one or more lacrimal and salivary glands, is described as `MIKULICZ'S DISEASE'. Typi­cal cases appear to be quite uncommon and hence this presentation.


  Case Report Top


P.M.K. a female patient aged 60 years, complained of slowly growing swellings of both the upper eye lids for the last 4 years. She had noticed gradual diminution of vision since 2 years. Pain in the eyes of 4 days duration brought her to the hospital.

Past history revealed that she had undergone an operation for the left eye lid tumour 3 years ago, but the swelling recurred with in a year.

General Examination revealed a smooth diffuse tumour of the left palate extending to the anterior pillars of the tonsil. Two inguinal lymph nodes, were just palpable on the right side. No other significant findings, were present.

Local Examination : A firm lobulated somewhat oval swelling about 10 cms. x 5cms. in size, was observ­ed over the right upper lid extending into the orbit. A similarly placed swelling about 8 x 5 cms was found on the left upper eye lid. There was blepharoptosis with proptosis down and inwards on both sides. Chemosis of both the conjunctivae with exposure kera­titis of lower part of the right cornea, was there. Both the swellings were free from bone and skin, except for a point of adhesion at the upper end of lateral orbital margin. Preauricular lymphnodes, were not palpable. Vision in right eye, was counting fingers 1 foot and in left eye counting fingers 10 feet improving to 6/36 with pin hole. Projection and pupil reaction to light, were normal.

Investigations

Haemoglobin -86%. Blood Group `O'. Total white blood cell count 7700/c.mm. Differential count -58% polymorphs, 38% lymphocytes and 4% eosinophils. No immature white blood cells, were detected. Montoux test was positive. V.D.R.L. was non reactive. X-ray of chest and skull, were normal. Biopsy of the inguinal lymphnode showed no evidence of any neoplasm or any other pathology.

Diagnosis

Provisional diagnosis of Mikulicz's disease, was made when no other cause was found. Both the tum­our masses, were enucleated in two separate sittings.

The tumours were found to be firm and lobulated with a thin capsule and were not infiltrating any other surro­unding structures but extending deep into orbit.

Patient refused surgery of the palatal tumour. Re­covery was uneventful. Vision improved to 6/36 in right eye and 6/12 in left eye with correction at the time of discharge.

During the follow up for a period of ten months, there has been no evidence of recurrence of the lid tumour whereas the tumour of the palate was found to be a little larger.

Histopathological Examination

Sections showed a thin fibrous capsule to the tumour mass, which was composed of sheets of small lymphocytes. There were no lymphoid follicles. Amongest the sheets of lymphocytes, were found scat­tered groups of ducts lined with low columnar cells.

Diagnosis : Mikulicz's Disease.


  Discussion Top


The Mikulicz's disease is one of the un­common conditions in which the lacrimal glands, are affected first followed at varying intervals of the salivary glands. There is slow pain less enlargement of the glands without signs of inflammation or consititutional distur­bances. Enlargement of lacrimal glands pro­duces ptosis. The present case is in line with classical descriptions. Difficulty in opening the mouth, is described as one of the manifes­tations, which was not a feature of the present case. This may be due to the more common involvement of partoid or submandibular sali­vary glands. In our case, the accessary gland of the palate, was affected. The palate swell­ing, was noted 3 years after the onset of the lid swelling. Tendency to recur is feature of this disease.

The exact nature of the lesion, is not defin­ed. Some workers consider it as a low grade malignancy andd even metastatis have been recorded though quite rarely. [2] Evans [3] prefers to call the tumour as "Benign lymphoepithelial lesion" and considers them as non neoplastic tumefactions of the lymphoglandular tissue while Morgan and Castleman° consider it as merely a manifestation of Sjogren's syndrome.

Simultaneous enlargements of salivary and lacrimal glands have been observed in some of the treatable granulomatus lesions like syphilis, tuberculosis and sarcoidosis as secondary manifestations. [2] Reticulosis such as Hodgkin's disease, may manifest as enlargement of these glands [1],[4],[6] . Such lesions have been accommo. dated under Mikulicz's syndrome. One should rule out the above mentioned conditions before coming to the diagnosis of Mikulicz's disease, where the etiology is obscure and appears primarily in the glandular tissues.

Although the disease is of chronic nature, it is mostly benign in its course.

Steroid therapy covered by an antibiotic such as streptomycin is worth trial. [2] However, the available treatment at present for this dis. ease, appears only to be surgery.


  Summary Top


A case of Mikulicz's disease, giving rise to primary enlargement of both lacrimal glands and minor salivary gland of the palate is reported.


  Acknowledgments Top


We thank very sincerely, the Superintendent of the Civil Hospital, Belgaum, for permitting us to publish this case. We are grateful to the Dean and Principal of J.N. Medical College, Belgaum, for their encouragement in preparation of this article.

 
  References Top

1.
Azzopardi. J.G., 1971, J. Clin. Path. 24, 744.   Back to cited text no. 1
    
2.
Dorrell, E.W., 1972 Modern Ophthamology Il. Vol. 4, 865, Edited by Arnold Sorsby. Butter Worth and Co., London.   Back to cited text no. 2
    
3.
Evans, R.W., 1968, Histological appearance of tumours. II, 939. E. and S. Livingstone Ltd., Edinburough & London.  Back to cited text no. 3
    
4.
Habibullah, C.M., 1969, J. Asso. Physicians India, 17, 667.  Back to cited text no. 4
    
5.
Morgan, W.S. and Castleman, B. 1953, Amer, J. Path., 29, 471.  Back to cited text no. 5
    
6.
Pinkus, G. S., 1970, Cancer, 25, 121.  Back to cited text no. 6
    


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