Haab's degeneration (Biber-Haab-Dimmer degeneration)

A localised form of amyloidosis with onset early in life (20-30 years of age), affecting both sexes. It is manifested by progressive corneal opacity with distinct borders and a network of lattice-like branching filaments. 

Haab's degeneration

Round spots may be scattered throughout the cornea, but the intervening spaces are clear, and there may be extracellular deposits of amyloid material. Visual function usually remains fairly satisfactory for long time. Aetiology unknown. Inheritance is autosomal dominant.

More details: 📖 Neuro-Ophthalmology Illustrated

Otto Haab (1850 – 1931) was a Swiss ophthalmologist.

Otto Haab

References

1: JONES ST, ZIMMERMAN LE. Macular dystrophy of the cornea (Groenouw type II);

clinicopathologic report of two cases with comments concerning its differential

diagnosis from lattice dystrophy (Biber-Haab-Dimmer). Am J Ophthalmol. 1959

Jan;47(1 Part 1):1-16. PMID: 13617346.

2: Szwarcowa C. Zwyrodnienie siatkowate rogówki Bibra-Haaba-Dimmera [Reticular

degeneration of cornea (Biber-Haab-Dimmer)]. Klin Oczna. 1977 Oct;47(10):449-50.

Polish. PMID: 303724.

3: Frezzotti R, Bonanni R. Appunti iconografici sulla degenerazione reticolare

familiare della cornea (Biber-Haab-Dimmer) [Iconographic data on familial

reticular degeneration of the cornea (Biber-Haab-Dimmer)]. Boll Ocul. 1966

Dec;45(12):907-9. Italian. PMID: 5999520.