Neuro-vascular Abnormalities with a Metameric Nevus: A Case Report and Review of Cobb Syndrome

Cobb syndrome, also known as cutaneomeningospinal angiomatosis, first described by Berenbruch in 1890 and reported by Cobb in 1915, is a rare disorder characterized by spinal vascular malformations and a corresponding dermatomal vascular nevus. It usually presents in children and adults as lower extremity weakness, back pain, sensory loss below the affected level, or paralysis. Onset may be acute, gradual, or follow a stuttering course. Symptoms are thought to result from rupture or expansion of the angioma leading to direct compression of the spinal cord or ischemia due to diverted blood flow. We report a case of a 29-year-old female with a constellation of congenital neuro-vascular abnormalities who presented with lower extremity paresthesias and urinary retention and was found to have a portwine stain in the S1 and S2 dermatomes without a corresponding spinal angioma on imaging.