Lymphomatoid papulosis with antigen deletion and clonal rearrangement in a 4-year-old boy

Lymphomatoid papulosis (LyP) is rarely seen in children. We report a case of LyP in a 4-year-old boy in whom immunopathologic studies demonstrated T cell antigen deletions. In contrast to all but two previous reports, a T suppressor (CD-8) phenotype was predominant. Southern blot analysis of DNA isolated from a typical skin lesion indicated a clonal rearrangement of the T cell receptor beta gene. Because of a 10% frequency of malignant lymphomas in patients with LyP, long-term observation is crucial, especially in children. We recommend routine clonal rearrangement studies for aid in diagnosis and follow-up, and as possible prognostic indicators in children with this condition.