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

• Published in: Journal of the American Academy of Dermatology Vol. 61; no. 6; pp. 993 - 1000
• Main Authors: de Souza, Aieska, MD, Camilleri, Michael J., MD, Wada, David A., MD, Appert, David L., MD, Gibson, Lawrence E., MD, el-Azhary, Rokea A., MD, PhD
• Format: Journal Article
• Language: English
• Published: United States Mosby, Inc 01.12.2009
• Subjects: Adolescent; CD8 Antigens - analysis; CD8 disorders; Child; Child, Preschool; Dermatology; Female; Gene Rearrangement, T-Lymphocyte; Humans; immunophenotypic features; Immunophenotyping; Infant; lymphomatoid papulosis; Lymphomatoid Papulosis - genetics; Lymphomatoid Papulosis - immunology; Lymphomatoid Papulosis - pathology; Male; pediatric patients; Skin Neoplasms - genetics; Skin Neoplasms - immunology; Skin Neoplasms - pathology; T-cell receptor gene rearrangement; LyP; World Health Organization; EORTC; lymphomatoid papulosis; immunophenotypic features; T-cell intracytoplasmic antigen; pediatric patients; T-cell receptor gene rearrangement; TCRGR; TIA-1; CD8 disorders; European Organization for Research and Treatment of Cancer; WHO
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/j.jaad.2009.05.014

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.