You are in: eMedicine Specialties > Dermatology > DISEASES OF THE VESSELS Cobb SyndromeArticle Last Updated: May 9, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 10
  Author: Christopher Norwood, MD, MS, Assistant Clinical Professor, Department of Dermatology, University of Connecticut Health Center Christopher Norwood 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 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 Editors: 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; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Rosalie Elenitsas, MD, Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center Author and Editor Disclosure Synonyms and related keywords: cutaneomeningospinal angiomatosis INTRODUCTIONSection 2 of 10
  BackgroundCobb 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. PathophysiologyThe 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. FrequencyInternationalIn 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/MorbidityThe 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.
RaceNo racial predilection is known, although most reported cases have been in whites. SexCobb syndrome has a slight male predominance. AgeDisease onset is in childhood or adolescence. Recently, a report described a 5-month-old child with a T5-T12 hemangioma and paraparesis. CLINICALSection 3 of 10
HistoryPatients 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
CausesThough 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. DIFFERENTIALSSection 4 of 10
Angiokeratoma Corporis Diffusum (Fabry Syndrome) Herpes Zoster Infantile Hemangioma WORKUPSection 5 of 10
Imaging Studies
Procedures
TREATMENTSection 6 of 10
Surgical CarePatients should be referred to a neurosurgeon and an interventional radiologist for embolization5, 6 and decompression. Consultations
FOLLOW-UPSection 7 of 10
ComplicationsA possible complication if treatment is delayed is Foix-Alajouanine disease4 or subacute necrotic myelopathy due to thrombosis in the spinal angioma. PrognosisThe prognosis was grim with paralysis inevitable and death from infection likely a century ago. Current intervention provides some hope for minimizing permanent neurological damage. The key is early diagnosis. MISCELLANEOUSSection 8 of 10
Medical/Legal PitfallsIn the past, patients were initially diagnosed with atypical poliomyelitis. Since early decompression can minimize symptoms, correct diagnosis is key. Patients with lower extremity weakness or pain should have a thorough skin examination and be examined with the proper imaging modalities promptly. MULTIMEDIASection 9 of 10
REFERENCESSection 10 of 10
Article Last Updated: May 9, 2006 | |||||||||||||||||||||||||||||||||||
