[Repeated attacks of pain in the nipple of a pregnant woman. Unusual manifestation of Raynaud's phenomenon].

History And Findings: A 38-year-old woman, in her 13th week of pregnancy, presented for angiological investigation of repeated episodes of pain in the right index finger. She also reported painful paleness of the right nipple evoked particularly by cold. This was her fifth pregnancy; she had miscarried twice. The nipple pain occurred spasmodically and in three phases, while the left nipple was unaffected. The attacks lasted c. 15 min, progressing from an ischemic to a hyperemic phase, accompanied by unbearable pain. When first examined there was circumscribed, purplish network-patterned discoloration of the right hand with blackened discoloration of the distal nail-bed of the index finger.

Investigations: Duplex-sonography revealed patency of the large arteries of the arm and hand. Transesophageal echocardiography showed a persistent foramen ovale and echo-density suspected to be a right atrial thrombus. Laboratory tests demonstrated anticardiolipin antibodies, suggesting an antiphospholipid syndrome.

Treatment And Course: On administration of low-molecular heparin, its dosage weight-adjusted, and 100 mg aspirin daily the discoloration of the finger quickly disappeared, but Raynaud's syndrome remained unchanged until she had stopped breast feeding (left breast only) after 8 months. A healthy girl had been delivered by cesarean section in the 39th week, weighing 3100 g. There were no further thromboembolic complications.

Conclusion: This very rare case of Raynaud's phenomenon in a nipple was associated with an anti-phospholipid syndrome.