In search of prognostic indicators for lymphomatoid papulosis: A retrospective study of 123 patients

• Published in: Journal of the American Academy of Dermatology 2012-06, Vol.66 (6), p.928-937
• Main Authors: de Souza, Aieska, MD, el-Azhary, Rokea A., MD, PhD, Camilleri, Michael J., MD, Wada, David A., MD, Appert, David L., MD, Gibson, Lawrence E., MD
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Biological and medical sciences; CD30 lymphoproliferative disorder; Cell Transformation, Neoplastic - genetics; Cell Transformation, Neoplastic - immunology; Cell Transformation, Neoplastic - pathology; chemokines and chemokine receptors; Child; Child, Preschool; cutaneous lymphomas; Dermatology; Disease Progression; Female; Gene Expression Profiling; Gene Rearrangement, T-Lymphocyte; Humans; lymphomatoid papulosis; Lymphomatoid Papulosis - genetics; Lymphomatoid Papulosis - immunology; Male; Medical sciences; Middle Aged; Prognosis; Receptors, Antigen, T-Cell - genetics; Retrospective Studies; Skin involvement in other diseases. Miscellaneous. General aspects; Skin Neoplasms - genetics; Skin Neoplasms - immunology; T-cell gene rearrangement; Young Adult
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/j.jaad.2011.07.012

Background Lymphomatoid papulosis (LyP) is a benign recurrent papulonodular skin eruption with histologically malignant features that sometimes (10%-20%) progresses to lymphoma. Objective We retrospectively evaluated the clinical course of patients with LyP and identify prognostic factors possibly indicating a malignant course. Methods Clinical, histopathologic, and immunologic features and molecular genetics were examined and correlated with clinical course and outcomes. Immunophenotyping and chemokine profiling were performed in select skin biopsy samples. A follow-up questionnaire was sent to patients. Clinical course and association with neoplastic disorders were correlated with LyP subtypes, molecular genetics, and immunophenotyping studies. Results Of 123 patients with LyP (1991-2008) followed up a mean of 4 years (range, 2 months to 14 years), 17 (14%) had an associated hematologic malignancy, 8 of which were mycosis fungoides. Histopathologic analyses demonstrated classic LyP type A (n = 69), B (n = 13), or C (n = 6), and a slight predominance of T-cell CD8 marker expression for type A. More than one type of lesion was present in 9 patients with a higher incidence of hematologic malignancies. T-cell receptor gene rearrangement positivity was about two times higher, with LyP associated with hematologic malignancy (82% vs 44%; odds ratio 5.7; P  = .02). Chemokine studies in a subset of 25 patients showed chemokine receptor (CCR) CCR4+ and thymus and activation-related chemokine (TARC+ ) in all LyP types and CCR3+ and chemokine-related receptor (CXCR) CXCR3+ in types B and C. Limitations Retrospective study design is a limitation. Conclusions Positive T-cell receptor gene rearrangement or diagnosis of mixed-type LyP may be a prognostic indicator of disease more prone to progress to lymphoma.