Muller and retinal pigment epithelium cells normally release immunosuppressive factors, but certain combinations of cytokines may stimulate retinal pigment epithelium cells to release factors encouraging inflammation.Generally, mild intraocular inflammation results in a small, discrete, evanescent lesions. Larger dots, having less discrete borders, are the result of high intensity intraocular inflammation. In essence, an immune response with the normal amount and appropriate cytokine release will result in small white dots and a misregulated response eventually will produce scarring of the retinal tissue. Treatment is required in the latter case to combat loss of vision. The white dots usually disappear naturally. Corticosteroids have been shown to speed up this process. The differences in the immune response of each patient may contribute to the differences seen between the white dot syndromes. Distinct diseases
Due to the number of common features among the multiple syndromes, many suggest that the white dot syndromes are not distinct and represent a spectrum of one disease. Gass described the AZOOR complex which consists of multiple evanescent white dot syndrome, nultifocal choroiditis, punctate inner choroiditis, acute idiopathic blind spot enlargement, acute macular neuroretinopathy, acute annular outer retinopathy, and acute zonal occult outer retinopathy. He suggested these diseases represent one disease due to common factors such as a high occurrence in females, unexplained visual field loss, and reduced electroretinographic amplitudes. Reddy et al. conducted a study on the blind spots in multifocal choroiditis, punctate inner choroiditis, multiple evanescent white dot syndrome, and diffuse subretinal fibrosis syndrome. Clinical and electroretinographic evidence suggested the diseases are distinct.
Pemphigus vegetans is a localized form of pemphigus vulgaris. in which there is a localized vegetating papillomatous response. The eroded areas do not heal like usual but form papillomatous growth and vegetation. Accounts for 1-2% of pemphigus cases and is a relatively benign variant of pemphigus vulgaris. Two forms are recognized:

Pemphigus vegetans of Neumann is a localized disease of pemphigus vulgaris slightly more extensive than pemphigus vegetans of Hallopeau. : 568  This type is more common and characterized by early lesions similar to Pemphigus Vulgaris with large bullae and erosive areas. Healing is through formation of granulation tissue. It is named for the Austrian Dermatologist, Isidor Neumann. Pemphigus vegetans of Hallopeau is a disease of localized pemphigus vulgaris. : 568  It is named for François Henri Hallopeau. This type is less aggressive and has pustules not bullae. These pustules heal by verrucous hyperkeratotic vegetations. See also
List of cutaneous conditions
Pemphigus
List of conditions caused by problems with junctional proteins

References


== External links ==