Clinical, histopathologic, and immunophenotypic features of lymphomatoid papulosis with CD8 predominance in 14 pediatric patients

Background

Lymphomatoid papulosis (LyP) is a cyclic papulonodular eruption that is clinically benign and histologically malignant. Association with hematologic neoplasias has been reported in 5% to 20% of all cases.

Objective

We sought to review the clinical and histopathologic features of LyP in pediatric patients.

Methods

We searched for the records of all patients with a clinical and histopathologic diagnosis of LyP seen at our clinic from January 1991 through April 2008. The cases of pediatric patients (aged < 20 years) were reviewed in detail.

Results

Of 123 patients with LyP identified, 14 (11%) were in the pediatric age group. Most were male (64%); mean age of onset was 12 years. Type A LyP was identified in 12 patients, one patient had type B, and none had type C (type not determined in one case). Ten cases showed CD8 predominance by immunohistochemistry. T-cell intracytoplasmic antigen staining was positive in 3 cases of CD8⁺ LyP type A and the one case of LyP type B. Lesional T-cell receptor gene rearrangement studies were negative in 9 of 10 patients with LyP type A. The average follow-up time was 5.5 years. Lesions improved with treatment in most cases, and none of the cases were associated with hematologic malignancies.

Limitations

This was a retrospective review.

Conclusions

Among our pediatric patients, we noted a predominance of CD8⁺ LyP, which does not seem to have an aggressive course. Further longitudinal studies are necessary to evaluate prognostic differences between CD4⁺ and CD8⁺ LyP and their biological significance.

Key words: CD8 disorders, immunophenotypic features, lymphomatoid papulosis, pediatric patients, T-cell receptor gene rearrangement

Abbreviations used: EORTC, European Organization for Research and Treatment of Cancer, LyP, lymphomatoid papulosis, TCRGR, T-cell receptor gene rearrangement, TIA-1, T-cell intracytoplasmic antigen, WHO, World Health Organization