CASE REPORTThe parents were descendants of the same great-grandparents (fourth generation). They denied knowledge of individuals in the family, other than their daughters, who had similar problems. Moreover, both parents and 5 of 9 maternal and paternal uncles and aunts were examined by us, and no additional cases were dliscovered. The pedigree and the HLA typing data (A, B, C, and DR) are shown in Figure 1. The HLA typing was performed on heparinized blood Figure 1, number 6), had limitation of movement of the wrists from birth. Later, she developed limitation of movement of the elbows, thumb deformities, an abnormal gait, and livedo reticularis of the legs. At the age of 12, she began to experience dyspnea on exertion. Pericarditis was diagnosed at another hospital; the condition did not respond to steroid therapy, and congestive heart failure developed. She underwent pericardiectomy at the age of 13, and the pathologic studies revealed chronic nonspecific pericarditis. When she presented to us, she had signs of congestive heart failure. She had flexion contractures of the interphalangeal joints of the thumbs, limited dorsiflexion of the wrists, and flexion contractures of the elbows and hips. The patient's asymptomatic siblings were called in for examination.Patient 2. This 14-year-old girl (Figure 1, number 7) had a history of trigger thumbs which resolved during her primary school years. At presentation, she had tenosynovitis of the wrists with limited dorsiflexion, flexion contractures of the elbows, limited internal rotation of the hips, synovitis of both knees, and limited plantar flexion of the ankles.Patient 3. This 13-year-old girl (Figure 1, number 8) exhibited camptodactyly of both fifth fingers and subluxation of the interphalangeal joint of the right thumb. In addition, she had limited movement of the wrists, flexion contractures of the elbows, limited movement of the hips, minimal synovitis of the knees, limited dorsiflexion of the feet, and livedo reticularis of the legs. Three years previously she had 2 bouts of acute pericarditis, separated by a few months. The
