COEUR D’ALENE, IDAHO – Juvenile linear scleroderma of the face – traditionally considered a disease limited to the skin – now is more properly viewed as a neurocutaneous disorder, Dr. Francesco Zulian said in his Sidney Hurwitz Memorial Lecture at the annual meeting of the Society for Pediatric Dermatology.
"We need acute neurologic screening at onset of juvenile linear scleroderma of the face and careful monitoring during follow-up," according to Dr. Zulian, chief of pediatric rheumatology at the University of Padua (Italy).
Dr. Francesco Zulian
In his series of 77 patients with juvenile linear scleroderma of the face, 46 (60%) proved to have CNS involvement based upon positive brain MRI or EEG findings. The MRI was positive in 73% of patients with neurologic symptoms, which included seizures, chronic headache, cranial nerve palsy, hemiparesis, and behavioral abnormalities.
Moreover, one-quarter of the 24 neurologically asymptomatic patients had organic brain lesions, as evidenced by multiple white and gray matter involvement on MRI. Similarly, 27% of neurologically asymptomatic patients had abnormal EEG findings.
"I am thinking – and other people agree with me – that probably we are dealing with a neurocutaneous condition," he said.
The onset of localized scleroderma of the face occurred at an average age of 5.3 years, which is 2 years earlier than disease onset in patients with juvenile localized scleroderma at other sites. Patients with linear scleroderma of the face who developed neurologic symptoms did so within 5 years of skin disease onset.
How best to monitor patients with juvenile linear scleroderma of the face for occult brain involvement remains an unresolved issue. MRI is expensive and requires sedation in children. Dr. Zulian has turned instead to cone beam CT, a relatively new imaging technique used in maxillofacial surgery and dentistry. It differs from standard CT in that it uses less radiation, takes less time, doesn’t involve sedation, and costs significantly less than MRI or conventional CT. These advantages permit more frequent patient monitoring.
Cone beam CT allows for the creation of three-dimensional images so that physicians can calculate the size of soft tissue and bone abnormalities and monitor their changes over time. Currently, Dr. Zulian is evaluating the utility of this imaging method in a prospective study involving 15 patients. He said he plans to complete the study and present the findings next year.
Dr. Zulian reported having no relevant financial conflicts.