Raynaud's phenomenon, anticentromere antibodies, and digital necrosis without sclerodactyly: an entity independent of scleroderma?
We describe 4 women of 43, 73, 84, and 86 years with Raynaud's phenomenon, severe digital necrosis, and high serum levels of anticentromere antibodies without skin thickening or internal organ sclerosis. Investigations revealed no diabetes or arterial vascular disease leading to arterial obstruction. Histologic examination did not show any dermal sclerosis or calcinosis. The intravenous infusions of prostaglandin reversed the ischemic lesions in 3 patients. These cases suggest that the triad Raynaud's phenomenon, anticentromere antibodies and necrosis of digits without sclerodactyly and sclerosis of internal organ should be considered as an entity distinct from scleroderma with sclerosis. For this entity we propose the name RACAND syndrome.
Sachsenberg-Studer
E M
EM
Department of Dermatology, J-W Goethe University Hospital, Frankfurt, Germany.
Prins
C
C
Saurat
J H
JH
Salomon
D
D
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D002503:Centromere / Q000276:immunology*; D003937:Diagnosis, Differential; D005260:Female; D005385:Fingers / Q000473:pathology*; D006801:Humans; D009336:Necrosis; D011928:Raynaud Disease / Q000097:blood / Q000175:diagnosis*; D012594:Scleroderma, Localized / Q000097:blood / Q000175:diagnosis*; D013577:Syndrome
D001323:Autoantibodies