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Lymphomatoid Papulosis

  • Reference work entry
  • First Online:
Dermatopathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 1495 Accesses

Definition

LyP is a primary cutaneous lymphoproliferative disorder that clinically manifests as relapsing multiple, self-regressing papulonodular lesions and is histologically characterized by atypical large CD30+ lymphoid cells. Together with primary cutaneous anaplastic large-cell lymphoma (PCALCL), LyP belongs to the spectrum of primary cutaneous CD30+ lymphoproliferative disorders according to the WHO-EORTC classification for primary cutaneous lymphomas and the WHO classification for tumors of hematopoietic and lymphoid tissues (2008, 4th edition).

Clinical Features

  • Incidence

    The estimated prevalence is 1–2 cases per 1,000,000 inhabitants.

  • Age

    LyP may affect patients at all ages but occurs mostly in the fourth and fifth decade. Children may also be affected.

  • Sex

    The male to female ratio is 1.5:1.

  • Site

    LyP may involve all body regions. The buttocks and the extremities are predilection sites. LyP lesions are only rarely found in the head and neck region. Involvement of the oral...

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References and Further Reading

  • Bekkenk, M. W., et al. (2000). Primary and secondary cutaneous CD30+ lymphoproliferative disorders: A report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood, 95, 3653–3661.

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  • Drews, R., Samel, A., & Kadin, M. E. (2000). Lymphomatoid papulosis and anaplastic large cell lymphomas of the skin. Seminars in Cutaneous Medicine and Surgery, 19, 109–117.

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  • Karai, L. J., Kadin, M. E., Hsi, E. D., Sluzevich, J. C., Ketterling, R. P., Knudson, R. A., & Feldman, A. L. (2013). Chromosomal rearrangements of 6p25.3 define a new subtype of lymphomatoid papulosis. American Journal of Surgical Pathology, in press.

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  • Kempf, W. (2006). CD30+ lymphoproliferative disorders: Histopathology, differential diagnosis, new variants, and simulators. Journal of Cutaneous Pathology, 33(Suppl 1), 58–70.

    Article  PubMed  Google Scholar 

  • Kempf, W., Levi, E., Kamarashev, J., Kutzner, H., Pfeifer, W., Petrogiannis-Haliotis, T., Burg, G., & Kadin, M. E. (2002). Fascin expression in cutaneous CD30-positive lymphoproliferative disorders. Journal of Cutaneous Pathology, 29, 295–300.

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  • Kempf, W., Pfaltz, K., Vermeer, M. H., et al. (2011), EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Blood, 118, 4024–4035.

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  • Kempf, W., Kazakov, D.V., Schärer, L., Rütten, A., Mentzel, T., Paredes, B. E., Palmedo, G., Panizzon, R. G., & Kutzner, H. (2013). Angioinvasive lymphomatoid papulosis: a new variant simulating aggressive lymphomas. American Journal of Surgical Pathology, 37, 1–13.

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  • Macaulay, W. L. (1968). Lymphomatoid papulosis. A continuing self-healing eruption, clinically benign–histologically malignant. Archives of Dermatology, 97, 23–30.

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  • Saggini, A., Gulia, A., Argenyi, Z., Fink-Puches, R., Lissia, A., Magaña, M., Requena, L., Simonitsch, I., & Cerroni, L. (2010). A variant of lymphomatoid papulosis simulating primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma. Description of 9 cases. American Journal of Surgical Pathology, 34, 1168–1175.

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  • Willemze, R., Meyer, C. J., Van Vloten, W. A., & Scheffer, E. (1982). The clinical and histological spectrum of lymphomatoid papulosis. British Journal of Dermatology, 107, 131–144.

    Article  CAS  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Kempf und Pfaltz, Histologische Diagnostik, Zürich, Switzerland

    Werner Kempf

  2. Department of Dermatology, University Hospital Zürich, Zürich, CH-8042, Switzerland

    Werner Kempf

Authors
  1. Werner Kempf
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Corresponding author

Correspondence to Werner Kempf .

Editor information

Editors and Affiliations

  1. Division of Pathological Anatomy, Department of Surgery and Translational Medicine, University of Florence, School of Human Health Sciences, Florence, Italy

    Daniela Massi

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© 2016 Springer International Publishing Switzerland

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Kempf, W. (2016). Lymphomatoid Papulosis. In: Massi, D. (eds) Dermatopathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-30006-1_445

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  • DOI: https://doi.org/10.1007/978-3-319-30006-1_445

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-30005-4

  • Online ISBN: 978-3-319-30006-1

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