Cobb Syndrome 

  • Author: Stephen J Krivda, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Nov 21, 2011
 

Background

Cobb syndrome is a rare, noninherited disorder that involves the association of spinal angiomas or arteriovenous malformations (AVM) with congenital, cutaneous vascular lesions in the same dermatome. Berenbruch first described the disorder in 1890, but it was not widely known until Cobb's report in 1915.[1] The importance of this syndrome is the recognition that cutaneous vascular lesions may hint at an accompanying spinal cord angioma or AVM that may result in weakness or paralysis.

Refer to the image below.

Cutaneous angioma. Courtesy of L. Cooke, MD. Cutaneous angioma. Courtesy of L. Cooke, MD.

Cobb's patient initially had been presumed to have poliomyelitis; however, Cushing deduced that a spinal cord lesion was present by the complete paralysis with definite upper level of anesthesia, priapism, visceral paralysis, and exaggerated reflexes. He even suggested that an angioma was the cause by noting a similarity to meningeal angioma with facial port-wine stain (Sturge-Weber syndrome).

Patients originally were treated with laminectomy/decompression as attempted ligation of the vascular malformations resulted in death by hemorrhage. Therapeutic radiation later was attempted with moderate success.

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Pathophysiology

The cutaneous manifestations of Cobb syndrome typically are present as port-wine stains (PWS) or angiomas (see image below), but reports exist of angiokeratomas,[2] angiolipomas, and lymphangioma circumscriptum.[3] The intraspinal lesions may be angiomas or AVMs. One case report exists of a patient with Cobb syndrome who had brain angiomas in addition to the classic lesions.

Cutaneous angioma. Courtesy of L. Cooke, MD. Cutaneous angioma. Courtesy of L. Cooke, MD.
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Epidemiology

Frequency

International

In the world literature, only 35 cases of Cobb syndrome are reported. The actual incidence may be higher as only symptomatic cases are diagnosed. In a 1927 study, autopsy findings showed that approximately 12% of cadavers had angiomas; these angiomas had been asymptomatic during life.

Mortality/Morbidity

The major debility from Cobb syndrome is the onset of weakness, paresis, sensory loss, and loss of bowel and bladder control. Patients generally experience the sudden onset of pain and weakness as children or young adults. These symptoms may remit or remain stable; however, they do tend to worsen over time either by discrete steps or continuously.

  • Paralysis may lead to early reporting; treatment can lessen the degree of impairment.
  • Early development of weakness may portend a more aggressive course.
  • A possible complication if treatment is delayed is Foix-Alajouanine disease[4] or subacute necrotic myelopathy due to thrombosis in the spinal angioma.

Race

No racial predilection is known, although most reported cases have been in whites.

Sex

Cobb syndrome has a slight male predominance.

Age

Disease onset is in childhood or adolescence. Recently, a report described a 5-month-old child with a T5-T12 hemangioma and paraparesis.

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Contributor Information and Disclosures
Author

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, American Society of Dermatopathology, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

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, American Medical Association, National Psoriasis Foundation, Phi Beta Kappa, Sigma Xi, and Women's Dermatologic Society

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Merck Salary Management position; Abbott Honoraria Speaking and teaching; Amgen Honoraria Review panel membership; Centocor Honoraria Consulting; Leo Consulting; Merck None Other

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

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

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD  Herman Beerman Associate 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 and American Society of Dermatopathology

Disclosure: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Christopher Norwood, MD, MS, to the development and writing of this article.

References

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

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

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

  4. 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.

  5. 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].

  6. Miyatake S, Kikuchi H, Koide T, Yamagata S, Nagata I, Minami S, et al. Cobb's syndrome and its treatment with embolization. Case report. J Neurosurg. Mar 1990;72(3):497-9. [Medline].

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

  8. 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. Sep 2011;3(3):293-6. [Medline].

  9. Aminoff MJ, Logue V. Clinical features of spinal vascular malformations. Brain. Mar 1974;97(1):197-210. [Medline].

  10. Aminoff MJ, Logue V. The prognosis of patients with spinal vascular malformations. Brain. Mar 1974;97(1):211-8. [Medline].

  11. Brant AJ, James HE, Tung H. Cutaneomeningospinal angiomatosis (Cobb syndrome) with tethered cord. Pediatr Neurosurg. Feb 1999;30(2):93-5. [Medline].

  12. Doppman JL, Wirth FP Jr, Di Chiro G, Ommaya AK. Value of cutaneous angiomas in the arteriographic localization of spinal-cord arteriovenous malformations. N Engl J Med. Dec 25 1969;281(26):1440-4. [Medline].

  13. Jessen RT, Thompson S, Smith EB. Cobb syndrome. Arch Dermatol. Nov 1977;113(11):1587-90. [Medline].

  14. Kaplan P, Hollenberg RD, Fraser FC. A spinal arteriovenous malformation with hereditary cutaneous hemangiomas. Am J Dis Child. Dec 1976;130(12):1329-31. [Medline].

  15. Karshner R, Rand C, Reeves D. Epidural hemangioma associated with hemangioma of the vertebrae. Report of a case. Arch Surgery. 1939;39:942-51.

  16. Laredo JD, Reizine D, Bard M, Merland JJ. Vertebral hemangiomas: radiologic evaluation. Radiology. Oct 1986;161(1):183-9. [Medline].

  17. Mercer RD, Rothner AD, Cook SA, Alfidi RJ. The Cobb syndrome: association with hereditary cutaneous hemangiomas. Cleve Clin Q. 1978;45(2):237-40. [Medline].

  18. Wakabayashi Y, Isono M, Shimomura T, Tajima A, Terashi H, Asada Y, et al. Neurocutaneous vascular hamartomas mimicking Cobb syndrome. Case report. J Neurosurg. Jul 2000;93(1 Suppl):133-6. [Medline].

 
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Cutaneous angioma. Courtesy of L. Cooke, MD.
Cutaneous angioma. Courtesy of L. Cooke, MD.
Cutaneous angioma. Courtesy of L. Cooke, MD.
MRI of spinal angioma. Courtesy of L. Cooke, MD.
 
 
 
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