A 6 year old boy presents with brownish spots all over the body and nodules on iris as “marked by an arrow” in the picture below.This condition is a feature of?
B. Tuberous Sclerosis.
D. Albright syndrome.
The nodules seen on iris as shown in the picture above represents Lisch nodules.
- Also known as iris hamartoma, is a pigmented hamartomatous nodular aggregate of dendritic melanocytes affecting the iris.
- These nodules are found in neurofibromatosis type 1, and are present in greater than 94% of patients over the age of six.
- They are clear, yellow-brown, oval to round, dome-shaped papules that project from the surface of the iris.
- These nodules typically do not affect vision, but are very useful in diagnosis.They are detected by slit lamp examination.
- They are not found in neurofibromatosis type 2.
Neurofibromatosis type 1
- It is a multisystem genetic disorder that is characterized by cutaneous findings, most notably café-au-lait spots and axillary freckling ,lisch nodules,optic nerve glioma,skeletal dysplasias, and by the growth of both benign and malignant nervous system tumors, most notably benign neurofibromas.