Clinical, histopathologic, and immunophenotypic features of lymphomatoid papulosis with CD8 predominance in 14 pediatric patients
ABSTRACT 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.
We sought to review the clinical and histopathologic features of LyP in pediatric patients.
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.
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.
This was a retrospective review.
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.
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ABSTRACT: BackgroundT-cells with a γδ phenotype have been associated with aggressive lymphomas. Yet, inflammatory skin disorders and low grade lymphoproliferative disorders have rarely been described with a predominance γδ T cell infiltrate.Objective To review our experience and to determine the clinical relevance of γδ T-cell phenotype in lymphomatoid papulosis (LyP) and pityriasis lichenoides (PL).MethodsA retrospective dermatopathology file review looking for LyP and PL characterized by a γδ T-cell phenotype was performed. Clinical manifestations and course, histological features and molecular data were analyzed.ResultsSix of 16 cases of LyP and 4 of 23 cases diagnosed as PL during a 5 year period (2009-14) were identified. The median follow up for the whole group was 16 months (3– 64), showing in all cases an indolent clinical course.Conclusions The detection of a predominantly γδ T cell phenotype in papular lymphoid-rich infiltrates in the absence of other lesions is not associated with a clinically aggressive course. γδ T-cell-rich variants of LyP and PL may reflect a spectrum of related conditions. This is a single academic center retrospective chart review of a relatively small sample.This article is protected by copyright. All rights reserved.British Journal of Dermatology 08/2014; 172(2). DOI:10.1111/bjd.13364 · 4.10 Impact Factor
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ABSTRACT: Mycosis fungoides and lymphomatoid papulosis are the most prevalent pediatric cutaneous lymphoma. Children present with early stage mycosis fungoides and progression to late stage is rare. Skin directed therapy is appropriate. Lymphomatoid papulosis is benign but the histological infiltrate is similar to aggressive systemic T-lymphoma. A history of spontaneously remitting tumors is essential for correct diagnosis and to prevent treatment as systemic lymphoma. Expectant therapy is appropriate in children. There is an association with development of systemic lymphoma in up to 10% with lymphomatoid papulosis and follow-up is required into adulthood. Cutaneous B-cell lymphomas (CBCL) are rare in children. Marginal zone lymphoma is most frequent. It presents with erythematous dermal nodules. Systemic spread is exceptional. All other types of primary cutaneous lymphoma are exceedingly rare in children. It is vital that children are discussed at supraregional multi-disciplinary team with an expertise in cutaneous lymphoma to ensure accurate diagnosis and correct management.Expert Review of Dermatology 01/2014; 8(5). DOI:10.1586/17469872.2013.838041
Piel 11/2014; 29(9). DOI:10.1016/j.piel.2014.07.001