eMedicine Specialties > Dermatology > Diseases of the Vessels

Cobb Syndrome

Author: Christopher Norwood, MD, MS, Assistant Clinical Professor, Department of Dermatology, University of Connecticut Health Center
Coauthor(s): 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
Contributor Information and Disclosures

Updated: Dec 12, 2008

Introduction

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.

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.

Pathophysiology

The cutaneous manifestations of Cobb syndrome typically are present as port-wine stains (PWS) or angiomas, 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.

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 disease4 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.

Clinical

History

Patients typically present with sudden onset of back or lower extremity radicular pain associated with numbness that can be localized below a specific dermatome. Less commonly, weakness or bowel/bladder dysfunction may be presenting symptoms.

Physical

  • Cutaneous lesions may be distributed anywhere in the dermatome, from midline back to abdomen.
  • Midline back lesions, on rare occasions, are associated with spina bifida.
  • Unilateral lesions provide a clue to the location of the feeding artery in the spinal canal.
  • The cutaneous lesion may be very faint and may be more pronounced when the patient performs a Valsalva maneuver. The increased abdominal pressure causes preferential filling of the cutaneous angioma.
  • Neurological examination reveals weakness or paralysis and numbness or decreased sensation with a sharp upper cutoff.

Causes

Although Cobb syndrome is thought to be noninherited, two case reports exist of inherited cutaneous angiomas in patients with Cobb syndrome. The forebears had no clinical evidence of spinal lesions. It has been postulated that Cobb syndrome, Wyburn-Mason syndrome, and PHACE (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) syndrome are segmental vascular syndromes that differ only in the timing and target of an error in neural plate migration.5

More on Cobb Syndrome

Overview: Cobb Syndrome
Differential Diagnoses & Workup: Cobb Syndrome
Treatment & Medication: Cobb Syndrome
Follow-up: Cobb Syndrome
Multimedia: Cobb Syndrome
References
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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. Aminoff MJ, Logue V. Clinical features of spinal vascular malformations. Brain. Mar 1974;97(1):197-210. [Medline].

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

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

  11. 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].

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

  13. 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].

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

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

  16. 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].

  17. 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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Further Reading

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Keywords

cutaneomeningospinal angiomatosis

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Contributor Information and Disclosures

Author

Christopher Norwood, MD, MS, Assistant Clinical Professor, Department of Dermatology, University of Connecticut Health Center
Christopher Norwood, MD, MS is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, and American Society for Laser Medicine and Surgery
Disclosure: Nothing to disclose.

Coauthor(s)

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
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.

Medical Editor

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; Genentech Honoraria Consulting; Incyte Grant/research funds Other; Warner Chilcott Honoraria Consulting; Merck Salary Management position; Abbott  Speaking and teaching

Pharmacy Editor

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.

Managing Editor

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: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine 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, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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