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Lipoid proteinosis: A rare entity.

Abstract
Urbach-Wiethe syndrome or lipoid proteinosis is a rare autosomal recessive disorder characterized histologically by infiltration of Periodic acid Schiff-positive hyaline material in the skin, upper aerodigestive tract, eyelids, and internal organs. Classical clinical features include scarring of the skin, beaded eyelid papules (moniliform blepharosis) and laryngeal infiltration leading to hoarseness of voice. Lipoid proteinosis can lead to life-threatening conditions such as acute respiratory distress and seizures. Awareness among ophthalmologists about this rare entity is crucial for appropriate management of these patients.
AuthorsBipasha Mukherjee, Pratheeba N Devi
JournalIndian journal of ophthalmology (Indian J Ophthalmol) Vol. 63 Issue 8 Pg. 680-1 (Aug 2015) ISSN: 1998-3689 [Electronic] India
PMID26576529 (Publication Type: Case Reports, Journal Article)
Topics
  • Adult
  • Biopsy
  • Diagnosis, Differential
  • Eyelid Diseases (diagnosis)
  • Eyelids (pathology)
  • Humans
  • Lipoid Proteinosis of Urbach and Wiethe (diagnosis)
  • Male
  • Rare Diseases
  • Tomography, X-Ray Computed

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