Bilateral Palpebral Pseudotumoral Edema Revealing Systemic Amyloidosis: A Case Report
A. Denial *
Department of Adult Ophthalmology, 20 August 1953 Hospital, Ibn Rochd University Hospital Center, Casablanca, Morocco.
Y. Tahri
Department of Adult Ophthalmology, 20 August 1953 Hospital, Ibn Rochd University Hospital Center, Casablanca, Morocco.
H. Sokrat
Department of Adult Ophthalmology, 20 August 1953 Hospital, Ibn Rochd University Hospital Center, Casablanca, Morocco.
A. Sandali
Department of Adult Ophthalmology, 20 August 1953 Hospital, Ibn Rochd University Hospital Center, Casablanca, Morocco.
Y. Hidan
Department of Adult Ophthalmology, 20 August 1953 Hospital, Ibn Rochd University Hospital Center, Casablanca, Morocco.
M. R. Bentouhami
Department of Adult Ophthalmology, 20 August 1953 Hospital, Ibn Rochd University Hospital Center, Casablanca, Morocco.
A. Hammouche
Department of Adult Ophthalmology, 20 August 1953 Hospital, Ibn Rochd University Hospital Center, Casablanca, Morocco.
A. Mchachi
Department of Adult Ophthalmology, 20 August 1953 Hospital, Ibn Rochd University Hospital Center, Casablanca, Morocco.
L. Benhmidoune
Department of Adult Ophthalmology, 20 August 1953 Hospital, Ibn Rochd University Hospital Center, Casablanca, Morocco.
R. Rachid
Department of Adult Ophthalmology, 20 August 1953 Hospital, Ibn Rochd University Hospital Center, Casablanca, Morocco.
*Author to whom correspondence should be addressed.
Abstract
Background: Amyloidosis is a rare disorder characterized by extracellular deposition of insoluble fibrillar proteins in tissues and organs. Ocular involvement is uncommon but may affect several structures including the eyelids, conjunctiva, lacrimal gland, and orbit. Because clinical manifestations are often nonspecific, diagnosis may be delayed. We report a case of bilateral palpebral pseudotumoral edema revealing systemic amyloidosis.
Case Presentation: A 63-year-old patient presented with progressive bilateral periorbital swelling associated with bluish discoloration of the eyelids and conjunctival masses that had evolved for more than two years without any history of trauma. Several ophthalmological and dermatological consultations had been performed, and symptomatic treatments were prescribed without clinical improvement. Laboratory investigations revealed hypergammaglobulinemia on serum protein electrophoresis. Histopathological examination of an eyelid biopsy confirmed amyloid deposition. Further systemic evaluation revealed additional organ involvement consistent with systemic amyloidosis. The patient underwent chemotherapy and symptomatic treatment. Surgical excision of infiltrated eyelid tissue followed by reconstructive surgery was proposed but declined by the patient. Clinical follow-up showed improvement in ecchymosis and ocular comfort, although eyelid swelling persisted.
Conclusion: Palpebral amyloidosis is a rare condition that may represent the first manifestation of systemic amyloidosis. Early histopathological confirmation and comprehensive systemic evaluation are essential for accurate diagnosis and appropriate management.
Keywords: Amyloidosis, eyelid amyloidosis, ocular adnexal amyloidosis, periorbital edema