Cobb Syndrome Clinical Presentation
- Author: Kendall M Egan, MD, FAAD; Chief Editor: Dirk M Elston, MD more...
The cutaneous manifestations of Cobb syndrome typically present as port-wine stains (PWSs) in a dermatomal distribution on the trunk. Other cutaneous vascular malformations, including angiomas, angiokeratomas, angiolipomas, cavernous hemangiomas, and lymphangioma circumscriptum, have also been less frequently reported. The intraspinal lesions are most commonly arteriovenous malformations (AVMs).
Kyphoscoliosis may occur if the spinal lesion involves the vertebral bodies.
Vascular cutaneous lesions are in a dermatomal distribution. See the images below.
Midline back lesions may be associated with spina bifida.
The cutaneous vascular lesions may be subtle. These lesions may become more pronounced and more easily identifiable when the patient performs the Valsalva maneuver.
A thorough neurologic examination may show deficits, depending on the severity of the spinal pathology.
It has been suggested that Cobb syndrome, Sturge-Weber syndrome, and PHACE syndrome (posterior fossa, hemangioma or other vascular birthmark present either on the outside or inside, arterial defect in the head and or neck area, cardiac problems, eye problems) may have a similar somatic mutation in the neural crest or mesoderm during development. The timing of this mutation during development may determine which syndrome develops.
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