Unusual presentation of scleroderma: case report

The combination of neurological, cardiac, and pulmonary disease in patients with systemic sclerosis (SS) is rare. The aim of the study was to report a patient with SS who manifested with migraine without aura, Raynaud's phenomenon, pulmonary hypertension, and postural tachycardia syndrome (POTS), a combination which has not been previously reported.

The patient is a 53-year-old Caucasian woman with diffuse cutaneous SS who was treated with methotrexate and developed migraine without aura since the age of 6 years. Since age 31 years, she developed Raynaud's phenomenon with several attacks daily, which could be triggered by cold and also occurred in summer. At the age of 52 years, pulmonary fibrosis with secondary pulmonary hypertension was diagnosed and bosentan was administered. At the age of 53 years, POTS was diagnosed. The frequency of migraine was one per week at the age of 53 years.

This case shows that migraine can be the initial manifestation of diffuse cutaneous SS, followed by Raynaud's syndrome, pulmonary hypertension and POTS.