EyeRounds Online Atlas of Ophthalmology

Contributor:
William Charles Caccamise Sr,MD, Retired Clinical Asst Prof of Ophthal. U.of Rochester School of Medicine & Dentistry

Category: Neuro-ophthalmology
Diagnosis: Pringle's Disease ( adenoma sebaceum ) in patient with tuberose ( tuberous ) sclerosis ( Bourneville's Disease )

Image Comments:

Reference: Fitzpatrick et al, Dermatology in General Medicine, 2nd edition 1979: "Tuberous sclerosis is an uncommon neurocutaneous whose features appear during the early life of the patient. The classical triad of adenoma sebaceum ( photos 1 and 2 ), seizures and mental retardation may, in fact, be preceded by the appearance of white macules ." The macules may have been overlooked in this patient since she was examined in 1966. The first report by Harris was in the Br.J.Dermatol in 1966: Tuberous sclerosis with vitiligo. The 3rd photo reveals ungual fibromas. As in this patient, they are more frequently periungual rather than subungual. Lesions can be seen periungually on the 1st finger ( the thumb ) and the 4th finger.

Pringle' Disease ( adenoma sebaceum ) in a patient with Bourneville's Disease ( tuberose sclerosis )

periungual fibromas - thumb and 4th finger

 

 

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