Lymphomatoid Papulosis

Lymphomatoid Papulosis

C. Cameron Yin, MD, PhD

Lymphomatoid papulosis (LyP), type C. A dense lymphoid infiltrate fills the dermis and is composed of medium to large atypical cells with frequent mitoses image. This lesion spontaneously regressed.

Immunohistochemical study for CD30 in a case of LyP type C. Strong and uniform CD30(+) is characteristic of LyP and sheets of CD30(+) cells support type C. This lesion spontaneously regressed.



  • Lymphomatoid papulosis (LyP)


  • Primary cutaneous CD30(+) T-cell lymphoproliferative disorder

    • This term also includes cutaneous anaplastic large cell lymphoma (C-ALCL)


  • Chronic, self-healing and recurrent skin lesions characterized by erythematous papules/nodules on trunk and extremities

  • Composed of large atypical cells in marked inflammatory background

  • Initially described by Macaulay as “a continuing self-healing eruption, clinically benign, histologically malignant”



  • Suggested factors

    • Viral infection, reduced immunosurveillance

    • Chronic antigenic stimulation, direct oncogenic effect of immunosuppressive drugs

  • Outbreaks may be triggered by stress or illness

  • TNFR-associated factor-1 and cutaneous lymphocyte antigen (E-selectin ligand) are highly expressed in LyP



  • Age

    • Median: 45 years (wide age range, including children)

  • Gender

    • Male to female ratio = 2-3:1


  • Trunk and extremities most common

  • Genital and oral mucosa can be rarely involved


  • Papular, papulonodular, or nodular skin lesions at different stages of development

    • Clusters or disseminated; ± ulceration

  • Individual skin lesions spontaneously regress within 3-12 weeks

    • After resolution, superficial scars can remain; hypo-or hyperpigmented

  • Waxing and waning clinical course; can persist for decades

  • LyP usually remains confined to skin

    • Can disseminate to regional lymph nodes

    • Very rarely disseminates elsewhere


  • No specific therapy for most patients; follow-up with attention to skin lesion changes or development of lymphadenopathy

  • Therapy options include

    • Surgical removal ± irradiation or low-dose methotrexate for skin-restricted disease

    • Multiagent chemotherapy for extracutaneous lesions


  • Excellent

    • 10-year disease-specific survival of ˜ 100%

  • Spontaneous regression in > 40% of patients

  • 10-20% of patients develop a 2nd lymphoma

    • Mycosis fungoides (MF), C-ALCL, or classical Hodgkin lymphoma

  • LyP patients have increased risk for nonlymphoid cancers


Histologic Features

  • Typically wedge-shaped lesion involving dermis

  • Epidermis is usually sparsely infiltrated and often ulcerated

  • 4 histologic types have been recognized, which represent a spectrum of disease

    • Arbitrarily designated as A, B, C, and D

  • Type A is most common

    • Scattered large atypical Reed-Sternberg-like cells

    • Numerous inflammatory cells including small lymphocytes, histiocytes, neutrophils, and eosinophils

  • Type B is uncommon (< 10%)

    • Simulates MF with epidermotropism and band-like dermal infiltrate

      • Composed of small to medium-sized lymphoid cells with cerebriform nuclei

      • Cannot be distinguished from MF by histology or immunophenotyping alone

      • Unlike MF, type B LyP usually regresses spontaneously

  • Type C

    • Large clusters or sheets of large atypical lymphoid cells with relatively few admixed inflammatory cells

    • Cannot be distinguished from C-ALCL by histology or immunophenotyping alone

    • Type C LyP is smaller (usually < 10 mm) than C-ALCL and spontaneously regresses over time

  • Type D has been recently described

    • Characterized by marked epidermotropism and CD8(+)



  • Types A and C

    • Large atypical cells are CD30(+), ALK(-)

    • Small lymphocytes are T cells

      • CD2(+), CD3(+), CD5(+), CD7 often (-); CD4(+), CD8(-)

    • Frequent expression of cytotoxic proteins: TIA-1, granzyme B, &/or perforin

  • Type B: Small cells with cerebriform nuclei are CD3(+), CD4(+), CD8(-), CD30(-)

    • Occasionally LyP has CD8(+) immunophenotype

      • More common in children

  • Type D: Atypical lymphocytes are CD30(+), CD3(+), CD4(-), CD8(+)

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Lymphomatoid Papulosis

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