We describe a patient with multicentric reticulohistiocytosis who presented, during the second trimester of pregnancy, with symmetric polyarthritis, marked erythematous, pulsatile synovial swelling of the distal interphalangeal joints of both hands, and widespread telangiectasias. She did not have the typical skin manifestations of multicentric reticulohistiocytosis. The erythema and pulsatility of the synovial swellings of the distal interphalangeal joints resolved after delivery, but she continued to have widespread active synovitis, which did not resolve until treatment with low-dose oral methotrexate was instituted. delivery. The destructive polyarthritis persisted after pregnancy, but she had an excellent, early response to treatment with low-dose oral methotrexate.
CASE REPORTThe patient. a 3.5-year-old East Timorese woman, presented to us in September I990 at 24 weeks gestation, with a I-month history of bleeding gums and facial rash, and a 3-month history of stiffness in the small joints ofthe hands and both knees and fatigue. A ganglion had been excised from the left wrist at another hospital 4 months before conception. Past obstetric history included I first-trimester spontaneous abortion and I successful pregnancy complicated by third-trimester gestational diabetes. She had no rash or joint symptoms during the previous pregnancies. There was no previous personal or family history to suggest a bleeding diathcsis.On physical examination at presentation. there were prominent telangiectasias over her face. oral mucosa and tongue, upper chest, and arms. She had marked erythematous synovial swelling over the DIP joints of both hands ( Figure I ) . On palpation. the synovial swellings were found to be pulsatile. and pulsed with the heart beat. There was mild tenderness over the joint lines. Effusions o f the proximal interphalangeal (PIP) joints of both hands and both knees were present, but without associated erythema or pulsation. There was a reducible flexion deformity of the right fifth DIP joint. There were no cutaneous nodular lesions. A soft systolic flow murmur was present, and uterine fundus height was compatible with pregnancy duration.Initial laboratory investigations revealed a mild