True about incontinenta pigmenti includes the following except:
INCONTINENTIA PIGMENTI X-linked dominant neurocutaneous syndrome with cutaneous, neurologic, ophthalmologic, and dental manifestations. Skin features occur in 4 stages Stage 1 (vesicular) Stage 2 (verrucous) Stage 3 (hyperpigmented) Stage 4 (atrophic/hypopigmented) Ophthalmologic findings Ophthalmologic findings occur in 20-35% of patients, and asymmetric involvement is common. Retinal vaso-occlusive events with resultant ischemia is primary mechanism in ocular pathology Treatment: Family counselling may prevent occurrence of new cases Skin lesions subside in adulthood spontaneously
X-linked dominant
Primary skin abnormality
Avascularity of peripheral retina
Ocular involvement is seen in almost 100% cases and is typically unilateral
Hyper Pigmented Disorders