A 23-year-old woman, who had
nonbullous congenital ichthyosiform erythroderma since her childhood, was diagnosed as
nephrotic syndrome caused by
systemic lupus erythematosus (SLE). She was pregnant but experienced fetal loss at the age of 25. Although 10 mg/day of oral
prednisolone was administered, low levels of serum
complement,
proteinuria,
thrombocytopenia (6.0 x 10(4)/mm3) and
biological false positive for STS continued. When she was 27 years old, right hemichorea developed. She was admitted to our hospital at the age of 28 because of low levels of serum
complement, high titers of anti
ds-DNA antibody, profuse
proteinuria, gingival
bleeding and
thrombocytopenia (1.5 x 10(4)/mm3). The
nephrotic syndrome gradually improved after 1 g/day of
methylprednisolone for 2 days and the oral
prednisolone dosage was then increased up to 40 mg/day, and was tapered to 10 mg/day. Epileptic attack (minor seizure) occurred at the age of 29. Continuous low levels of serum
complement and high titers of anti
ds-DNA antibody were improved by adding 50 mg/day of
cyclophosphamide. However, high levels of
beta 2 GPI dependent
anticardiolipin antibody and
lupus anticoagulant activity were observed throughout the study. Our patient was a very rare case of congenital
ichthyosis with typical
antiphospholipid antibody syndrome and SLE. A few cases of
acquired ichthyosis associated with SLE has been reported, and
ichthyosis developed only in active stage of SLE. However, our patient's ichthyosiform lesions were not changed throughout the course.