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Ophthalmology and Visual Sciences

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Posterior Polymorphous Corneal Dystrophy (PPMD)

Contributor: Jesse Vislisel, MD

Updated April 5, 2016

Posterior polymorphous corneal dystrophy (PPMD, PPCD) is a rare, bilateral, autosomal dominant inherited corneal dystrophy.  The corneal abnormality in PPMD occurs at the level of Descemet's membrane and endothelium, and rarely will result in corneal edema or elevated intraocular pressure. The three main patterns in which PPMD may present include endothelial vesicle-like lesions, band lesions, and diffuse deep stromal opacities.

For more information on PPMD read the related case report

Figure 1: Vesicle-like lesions in PPMD

Contributors: Jesse Vislisel, MD, Kenneth M. Goins, MD

Photographer:  Brice Critser, CRA

March 2, 2015

Vesicle-like lesions at the level of Descemet's membrane and endothelium are the hallmark lesions of PPMD.  They appear as transparent cystic lesions surrounded by gray halos and commonly occur in lines or clusters.  The figures below demonstrate the classic appearance of these lesions with direct illumination, retroillumination, and on specular microscopy.

transparent cystic lesions surrounded by gray halos and commonly occur in lines or clusters, direct illumination

transparent cystic lesions surrounded by gray halos and commonly occur in lines or clusters, retroillumination

transparent cystic lesions surrounded by gray halos and commonly occur in lines or clusters, specular microscopy


Figure 2: Band lesions in PPMD

Contributors: Jesse Vislisel, MD, Kenneth M. Goins, MD

Photographer:  Brice Critser, CRA

Band lesions, sometimes called "snail tracks," are classically horizontal lesions with parallel, scalloped, non-tapering edges at the level of the posterior cornea.  Below are examples of these lesions in 2 separate patients.

Patient 2a

Band lesions, sometimes called "snail tracks" Band lesions, sometimes called "snail tracks" Red Reflex: Band lesions, sometimes called "snail tracks" Slit lamp: Band lesions, sometimes called "snail tracks"

Patient 2b

Band lesions in posterior polymorphous corneal dystrophy

slit lamp, Band lesions in posterior polymorphous corneal dystrophy


Figure 3: Diffuse posterior stromal opacities in PPMD

Contributors: Jesse Vislisel, MD, Kenneth M. Goins, MD

Photographer:  Brice Critser, CRA

PPMD may present with diffuse, gray-white opacities at the level of Descemet's membrane.  There may be deep stromal haze adjacent to the lesions.  Below are slit lamp photographs of two patients with such lesions.

Patient 3a

Patient 1. diffuse, gray-white opacities at the level of Descemet's membrane

Patient 1. diffuse, gray-white opacities at the level of Descemet's membrane


Patient 3b

Patient 1. diffuse, gray-white opacities at the level of Descemet's membrane

patient 2. Posterior polymorphous corneal dystrophy (PPMD) may present with diffuse, gray-white opacities at the level of Descemet's membrane

patient 2. PPMD


Figure 4: Confocal microscopy of affected endothelium in PPMD

Contributor: Andrew Doan, MD, PhD, University of Iowa

Confocal microscopy of the endothelium in PPMD shows epithelial-like cells. Normal endothelial cells are hexagonal and flat.

posterior polymorphous dystrophy (PPMD)
(higher resolution image not available)


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last updated: 04/05/2016