Cutaneous T-cell lymphoma arising from parakeratosis variegata: long-term observation with monitoring of T-cell receptor gene rearrangements.

BACKGROUND

Parakeratosis variegata is a rare skin disease first described in 1890. Even today, the disease entity remains confusing because various names indicating similar skin conditions have been used. Several cases of parakeratosis variegata have been reported to develop into cutaneous T-cell lymphoma, but there have been no reports describing the occurrence of lymphoma after a long-term follow-up period nor have T-cell receptor gene rearrangements been monitored in this disease.

OBJECTIVE

Our purpose was to determine whether parakeratosis variegata (long-standing premycotic condition) can develop into cutaneous T-cell lymphoma.

METHODS

We analyzed skin specimens from a patient with a 33-year history of parakeratosis variegata by Southern blotting using a T-cell receptor gene probe.

RESULTS

We could detect apparent rearranged bands of T-cell receptor gene in the skin specimens taken in 1993 in contrast to the DNA analysis in 1988 which featured no such rearranged band.

CONCLUSION

This case represents a critical stage of parakeratosis variegata converting to lymphoma. Our results indicate that parakeratosis variegata generated monoclonality of T cells in its chronic course.