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Cobb Syndrome Clinical Presentation

  • Author: Kendall M Egan, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: May 10, 2016
 

History

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,[4] angiolipomas, cavernous hemangiomas,[5] and lymphangioma circumscriptum, have also been less frequently reported.[6] The intraspinal lesions are most commonly arteriovenous malformations (AVMs).

Kyphoscoliosis may occur if the spinal lesion involves the vertebral bodies.

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Physical

Vascular cutaneous lesions are in a dermatomal distribution. See the images below.

Cutaneous vascular lesions. Courtesy of L. Cooke, Cutaneous vascular lesions. Courtesy of L. Cooke, MD.
Cutaneous vascular lesions. Courtesy of L. Cooke, Cutaneous vascular lesions. Courtesy of L. Cooke, MD.

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.

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Causes

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.[7]

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Contributor Information and Disclosures
Author

Kendall M Egan, MD, FAAD Dermatologist, Veteran's Affairs Medical Center; Dermatologist, Spruce Health, Dermatologist, DermOne

Kendall M Egan, MD, FAAD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists

Disclosure: Nothing to disclose.

Coauthor(s)

Charles Egan, DO Staff Neuroradiologist, MRI Director, Naval Hospital Camp Lejeune

Charles Egan, DO is a member of the following medical societies: American College of Radiology, American Medical Association, American Roentgen Ray Society, American Society of Neuroradiology, Radiological Society of North America

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Stephen J Krivda, MD Assistant Professor of Dermatology, Uniformed Services University of the Health Sciences; Chief of the Integrated Department of Dermatology, Chief of Dermatology Service, Director of Dermatopathology, Staff Dermatopathologist, Walter Reed Army Medical Center; Head, Department of Dermatology, Staff Dermatologist and Dermatopathologist, National Naval Medical Center

Stephen J Krivda, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, American Society of Dermatopathology, Phi Beta Kappa

Disclosure: Nothing to disclose.

Abby S Van Voorhees, MD Assistant Professor, Director of Psoriasis Services and Phototherapy Units, Department of Dermatology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania

Abby S Van Voorhees, MD is a member of the following medical societies: American Academy of Dermatology, Women's Dermatologic Society, National Psoriasis Foundation, American Medical Association, Phi Beta Kappa, Sigma Xi

Disclosure: Received honoraria from Amgen for consulting; Received honoraria from Abbott for consulting; Partner received salary from Merck for management position; Received honoraria from Abbott for speaking and teaching; Received honoraria from Amgen for review panel membership; Received honoraria from Centocor for consulting; Received honoraria from Leo for consulting; Received none from Merck for other.

Acknowledgements

The views expressed in this presentation are the authors' and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense, or the US Government.

References
  1. Cobb S. Haemangioma of the spinal cord associated with skin naevi of the same metamere. Annals Surgery. 1915. 62:641-9.

  2. Abtahi-Naeini B, Saffaei A, Pourazizi M. Unusual cause of lower extremity wounds: Cobb syndrome. Int Wound J. 2015 Oct 1. [Medline].

  3. Pal P, Ray S, Chakraborty S, Dey S, Talukdar A. Cobb syndrome: A rare cause of paraplegia. Ann Neurosci. 2015 Jul. 22 (3):191-3. [Medline].

  4. Clinton TS, Cooke LM, Graham BS. Cobb syndrome associated with a verrucous (angiokeratomalike) vascular malformation. Cutis. 2003 Apr. 71(4):283-7. [Medline].

  5. Matsui Y, Mineharu Y, Satow T, Takebe N, Takeuchi E, Saiki M. Coexistence of multiple cavernous angiomas in the spinal cord and skin: a unique case of Cobb syndrome. J Neurosurg Spine. 2014 Feb. 20 (2):142-7. [Medline].

  6. Shim JH, Lee DW, Cho BK. A case of Cobb syndrome associated with lymphangioma circumscriptum. Dermatology. 1996. 193(1):45-7. [Medline].

  7. Krings T, Geibprasert S, Luo CB, Bhattacharya JJ, Alvarez H, Lasjaunias P. Segmental Neurovascular syndromes in children. Neuroimaging Clin N Am. May 2007. 17:245-58. [Medline].

  8. Jagla M, Szymonska I, Kruczek P. Sonographic findings in a neonate with Cobb syndrome. J Clin Ultrasound. 2013 May. 41(4):258-60. [Medline].

  9. Saraf-Lavi E et al. Detection of spinal dural arteriovenous fistulae with MR imaging and contrast-enhanced MR angiography: sensitivity, specificity, and prediction of vertebral level. ASNR AM J Neuroradiol. 2002. 23 (5):858-67.

  10. Dilmé-Carreras E, Iglesias-Sancho M, Márquez-Balbás G, Sola-Ortigosa J, Umbert-Millet P. Cobb syndrome: case report and review of the literature. Dermatology. 2010. 221 (2):110-2. [Medline].

  11. Spiotta AM, Hussain MS, Masaryk TJ, Krishnaney AA. Combined endovascular and surgical resection of a giant lumbosacral arteriovenous malformation in a patient with Cobb syndrome. J Neurointerv Surg. 2011 Sep. 3 (3):293-6. [Medline].

  12. Schirmer CM, Hwang SW, Riesenburger RI, Choi IS, David CA. Obliteration of a metameric spinal arteriovenous malformation (Cobb syndrome) using combined endovascular embolization and surgical excision. J Neurosurg Pediatr. 2012 Jul. 10(1):44-9. [Medline].

  13. Soeda A, Sakai N, Iihara K, Nagata I. Cobb syndrome in an infant: treatment with endovascular embolization and corticosteroid therapy: case report. Neurosurgery. 2003 Mar. 52(3):711-5; discussion 714-5. [Medline].

  14. Wirth FP, Post KD, Di Chiro G. Foix-Alajouanine disease. Spontaneous thrombosis of a spinal cord arteriovenous malformation: a case report. Neurology. 1970. 20:1114-18.

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Cutaneous vascular lesions. Courtesy of L. Cooke, MD.
Cutaneous vascular lesions. Courtesy of L. Cooke, MD.
Cutaneous vascular lesions. Courtesy of L. Cooke, MD.
MRI of spinal vascular lesion. Courtesy of L. Cooke, MD.
 
 
 
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