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Primary localised nodular amyloidosis of the eyelid and conjunctiva: a case report.

Sir, Amyloidosis is characterised by the deposition of abnormal fibrillar protein in the extracellular spaces of the body causing alterations of the tissue architecture and function. It may present as systemic amyloidosis, or it may be restricted to a single site as in localised amyloidosis with a variable clinical picture. We report an unusual case of a nodular amyloid eyelid tumour involving the conjunctiva without systemic amyloid involvement or myeloma. A 65-year-female presented to the ophthalmology outpatient department with a 1-year history of progressive painless thickening of the lower left eyelid. Physical examination revealed a healthy appearing woman. The patient’s visual acuity was 20/20 in OS with normal anterior segment. The lower lid was thickened with a firm, painless, nodular tumour extending onto and involving the conjunctiva (Fig. 1). There was no evidence of healed trachoma or any other chronic inflammatory process in either eye. There was no previous history of medical or surgical treatment or drug therapy or chemical burns. CT scan showed uniform thickening of the lower eyelid with no infiltration into the orbit. With a clinical differential diagnosis of chronic tarsitis, squamous cell carcinoma, or meibomian cell carcinoma, an incisional wedge biopsy was taken. Histologically, large nodular deposits of amorphous, eosinophilic, hyaline-like material was seen filling the entire papillary and reticular dermis and focally within the walls of deep dermal blood vessels and eccrine sweat gland coils. It involved both the eyelid and conjunctiva (Fig. 2). The overlying epidermis was thinned out with loss of rete pegs. At the periphery of the deposits many mature plasma cells were found admixed with a few lymphocytes and occasional giant cell. These deposits were congophilic with an apple green birefringence on polaroscopy and showed metachromasia with methyl violet. Immunohistochemical studies showed staining for kappa immunoglobulin light chains only, indicating that the amyloid deposits were derived from a monoclonal population of plasma cells or B cells. The scattered plasma cells within the deposits did not show monoclonality by kappa (k) or lambda (l) light chain immunohistochemical staining, which excluded a focal plasmacytoma. A work-up was negative for systemic amyloidosis including complete blood count, comprehensive metabolic panel, serum electrophoresis, sedimentation rate and liver function tests. Ultrasonography of the abdomen was unremarkable. Multiple myeloma was ruled out by the absence of Bence Jones proteins in urine, normal serum electrophoresis and unremarkable bone marrow examination. Chest radiography, echocardiogram and renal ultrasound were normal. Biopsy of buccal and rectal mucosa was negative for amyloid. Owing to the localised nature of the lesion, the patient opted for conservative management and is on continued follow-up. Cutaneous amyloid deposits may occur in systemic forms or may be localised to skin in the absence of other organ involvement. Systemic forms of amyloidosis that may exhibit cutaneous involvement include primary systemic amyloidosis, myeloma with systemic amyloidosis and certain hereditary syndromes such as familial amyloid polyneuropathy, familial Mediterranean fever and Muckle– Wells syndrome. Primary localised cutaneous amyloidosis (PLCA) includes macular amyloidosis, lichen amyloidosis and nodular amyloidosis. Amyloid deposits in nodular amyloidosis originate from immunoglobulin light chain (AL type). Both l and k light chain amino acid homologies have been found, with l chains being more common. However, our patient showed a k derivation. Fig. 1 Nodular amyloid tumour causing thickening of left lower eyelid. Inset shows involvement of conjunctiva also.