Etiology • Idiopathic/congenital • A proliferation of dermal melanocytes over a specific anatomic
distribution
Clinical Presentation • Macular, grayish blue discoloration of skin and mucosa over
the distribution of the ophthalmic and maxillary branches of the trigeminal nerve
• Unilateral distribution • Sclera on the involved side may be affected.
Microscopic Findings • Diffuse unencapsulated proliferation of spindle-shaped
distribution
Clinical Presentation • Macular, grayish blue discoloration of skin and mucosa over
the distribution of the ophthalmic and maxillary branches of the trigeminal nerve
• Unilateral distribution • Sclera on the involved side may be affected.
Microscopic Findings • Diffuse unencapsulated proliferation of spindle-shaped
melanocytes within dermis/submucosa, parallel to surface • Pigment production may be florid.
Diagnosis • Clinical presentation
Treatment • None • Cosmetic
Prognosis • Excellent