|Year : 2001 | Volume
| Issue : 2 | Page : 116-117
Conjunctival amyloidosis of both eyelids
Gabriel Rodrigues1, Vikram Sanghvi2, Murad Lala3
1 Department of Surgical Oncology, Tata Memorial Hospital, Mumbai, India
2 MS,FICS. Department of Surgical Oncology, Tata Memorial Hospital, Mumbai, India
3 MCH. Department of Surgical Oncology, Tata Memorial Hospital, Mumbai, India
MS,DNB. Department of Surgical Oncology, Tata Memorial Hospital, Mumbai, India
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Rodrigues G, Sanghvi V, Lala M. Conjunctival amyloidosis of both eyelids. Indian J Ophthalmol 2001;49:116-7
Amyloidosis of the palpebral conjunctiva is a rare condition which results in chronic ocular discomfort.[1-3] We present our experience with this rare patient in whom both upper and lower palpebral conjunctivae were involved, but no systemic amyloidosis.
Amyloidosis results from the deposition of insoluble, fibrous amyloid proteins, nearly always in the extracellular spaces of organs and tissues. With extensive deposits, the organ involved might attain tumorous proportions. Various treatment approaches including conservative surgical excision, [1, 2] cryotherapy and superficial radiotherapy have been attempted with partial success.
| Case report|| |
A 43-year-old man presented with a diffuse swelling of left lower eyelid for 12 years. This involved the upper eyelid in the last 6 months. Both palpebral conjunctivae were congested [Figure - 1]. The pathology was predominantly in the tarsal plates and the overlying skin was stretched. Detailed history did not reveal any preceeding chronic ophthalmic complaints. The patient had already undergone multiple biopsies elsewhere with inconclusive pathology reports. Transconjunctival biopsy at our hospital revealed deposition of acellular, pale, eosinophilic, hyaline material beneath the conjunctival epithelium [Figure - 2]. There was a sprinkling of mature plasma cells and lymphocytes. On congo red staining, the material exhibited congophilia, which on polarisation showed apple-green birefringence suggestive of amyloid deposition. The birefringence was sensitive to potassium permanganate pretreatment, signifying secondary amyloid deposition.
Immunoelectrophoresis revealed normal electrophoretic pattern, absence of monoclonal band, normal immunoglobulin levels (IgA, IgM, IgG) and absence of Bence-Jones proteins in the urine. Computerised tomography of the eye showed soft tissue mass in both the left eyelids with no intraorbital extension. Extraocular muscles, eyeball, retrobulcar area and optic nerve were normal. To rule out systemic amyloidosis, we performed urine protein, blood counts, ultrasound scan of abdomen and pelvis and echocardiogram, all of which were normal. The patient was advised surgery but after being made aware of the benign nature of the disease, and in view of the 12-year old history, he insisted that he be kept under observation. On follow-up, the lesion appeared to be static.
| Discussion|| |
Amyloidosis of the conjunctiva is rare.[1-3, 7,  This has previously been reported by a few authors as an unusual cause of ptosis and equally uncommon complication of trachoma. The term 'amyloidosis' was coined by Virchow in 1854 on the basis of the colour obtained upon staining with iodine and sulphuric acid. The prevalent classification of amyloidosis is based on clinical features, using the nomenclature recommended at the Sixth International Symposium on Amyloidosis in 1990.
Localised conjunctival amylodosis is apparently the most common non-familial ophthalmological manifestation of amyloidosis. The disease is usually unilateral as in our patient, but may involve both eyes. It usually begins in the fornix and extends into the bulbar and palpebral conjunctiva. Patients typically have a long history of slowly enlarging tumefaction of the eyelids with multiple biopsies and surgeries. In our patient too, he gave a past history of multiple biopsies. A review of literature showed that treatments varied from conservative local excision, [1, 2] cryotherapy to superficial cobalt therapy, [4, 7] but with partial success.
The disease is known to recur locally with persistent ocular complaints that could perhaps be attributed to conservative local excision and persistent disease. Systemic examination in all these patients was normal. Aetiology and pathogenesis of the disease is still unclear. Surgery is still the gold standard of management which should be as conservative as possible even though infrequent recurrences have been reported. [1, 2]
| References|| |
Hill VE, Brownstein S, Jordon DR. Ptosis secondary to amyloidosis of the tarsal conjunctiva and tarsus. Am J Ophthalmol
O'Donnell B, Wuebbolt G, Collin R. Amyloidosis of the conjunctiva. ANZ J Ophthalmol
Jain NS, Gupta AN. Amyloidosis of the conjunctiva. Br J Ophthalmol
Pecora JL, Sambursky JS, Vargha Z. Radiation therapy in amyloidosis of the eyelid and conjunctivae: A case report. Annals of Ophthalmol
Sipe JD, Cohen AS. Amyloidosis. In: Fauci AS, Braunwald E, Isselbacher KJ, Wilson JD, Martin JB, Kasper DL et al, editors. Harrison's Principles of Internal Medicine
. McGraw-Hill; 1998, vol.2, pp.1850-60.
Sandgren O. Ocular amyloidosis, with special reference to the hereditary forms with vitreous involvement. Surv Ophthalmol
Chumbley LC, Peacock OS. Amyloidosis of the conjunctiva - An unusual complication of trachoma: A case report. South Medical Journal
Smith ME, Zimmerman LE. Amyloidosis of the eyelid and conjunctiva. Arch of Ophthalmol
[Figure - 1], [Figure - 2]