Eyelid Lesions in Lipoid Proteinosis or Urbach-Wiethe Disease: Case Report and Review of the Literature

Autor:	Nuria Ibáñez-Flores, Josep Maria Sancho, Vanesa Cuadrado, Miquel Callizo, Jessica Laue, Xavier Graell
Rok vydání:	2011
Předmět:	Male medicine.medical_specialty Pathology Treatment outcome Clinical manifestation Urbach–Wiethe disease Risk Assessment Severity of Illness Index Rare Diseases Pathognomonic Surgical removal Biopsy medicine Humans medicine.diagnostic_test business.industry Biopsy Needle Middle Aged Usually progressive medicine.disease Immunohistochemistry Dermatology eye diseases Ophthalmology Treatment Outcome medicine.anatomical_structure Eyelid Diseases Lipoid Proteinosis of Urbach and Wiethe Laser Therapy sense organs Eyelid business Follow-Up Studies
Zdroj:	Orbit. 30:242-244
ISSN:	1744-5108 0167-6830
DOI:	10.3109/01676830.2011.579685
Popis:	Lipoid proteinosis (LP) or Urbach-Wiethe disease is a recessively inherited disorder not usually seen by ophthalmologists. It is characterized by non-inflammatory, persistent papules on the skin and mucous membranes. The first clinical manifestation of LP is usually progressive hoarseness. The lid lesions, consisting of beaded papules along the lid margins (moniliform blepharosis), are considered almost pathognomonic of the disease. The interesting clinical and histopathological features of LP are described in a 45-year-old man with a history of lipoid proteinosis, who presented to us for evaluation of ocular discomfort and unusual multiple eyelid lesions. We surgically removed all the eyelid lesions. The histologic findings were consistent with LP. Knowledge of the typical eyelid lesions may help to diagnose many typical or atypical cases of LP, although biopsy confirmation is always necessary. In our experience, the surgical removal of the eyelid lesions seems to be curative and due to the amount of eyelid lesions that can be developed, it is recommended to initiate an early treatment as soon as possible to avoid postoperative deformities.
Databáze:	OpenAIRE
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