A case of pityriasis rubra pilaris associated with unclassified connective tissue disease and sepsis in clinical course]

The patient was a 23-year-old woman with HTLV-I carrier and was diagnosed as pityriasis rubra pilaris soon after her birth. In November 1990 at her age of 16, she began to have fever and polyarthralgia which were not improved despite the administration of antibiotics. Her laboratory findings showed the positive antinuclear antibody and anti-RNP antibody suggesting one of collagen diseases. A tentative diagnosis as unclassified connective tissue disease (UCTD) was made since her symptoms and laboratory findings were not satisfied with any criteria for rheumatic disorders. The steroid therapy was started in February 1991 and showed a good response. On April 9th, 1996, however, she was admitted to our hospital because of recurrence of high fever and chills. This time, she had sepsis because of the evidence that Enterococcus faecalis was detected in blood culture. Although her condition was improved by antibiotics on June 4th 1996, high fever re-appeared and followed by convulsion and disseminated intravascular coagulation. After the doses of prednisolone per day was increased to 40 mg with antibiotics and anticoagulant, her condition gradually improved. This patient was a rare case of pityriasis rubra pilaris associated with UCTD and sepsis in clinical course.