Clinical, histopathologic, and immunophenotypic features of lymphomatoid papulosis with CD8 predominance in 14 pediatric patients - 24/08/11
Reprint requests: Rokea A. el-Azhary, MD, PhD, Department of Dermatology, Mayo Clinic, 200 First St SW, Rochester, MN 55905.Abstract |
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.
Le texte complet de cet article est disponible en PDF.Key words : CD8 disorders, immunophenotypic features, lymphomatoid papulosis, pediatric patients, T-cell receptor gene rearrangement
Abbreviations used : EORTC, LyP, TCRGR, TIA-1, WHO
Plan
| Funding sources: None. |
|
| Conflicts of interest: None declared. |
Vol 61 - N° 6
P. 993-1000 - décembre 2009 Retour au numéro
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