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.
TERMINOLOGY
Abbreviations
  • Lymphomatoid papulosis (LyP)
Synonyms
  • Primary cutaneous CD30(+) T-cell lymphoproliferative disorder
    • This term also includes cutaneous anaplastic large cell lymphoma (C-ALCL)
Definitions
  • 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”
ETIOLOGY/PATHOGENESIS
Unknown
  • 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
CLINICAL ISSUES
Epidemiology
  • Age
    • Median: 45 years (wide age range, including children)
  • Gender
    • Male to female ratio = 2-3:1
Site
  • Trunk and extremities most common
  • Genital and oral mucosa can be rarely involved
Presentation
  • 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
Treatment
  • 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
Prognosis
  • 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
MICROSCOPIC PATHOLOGY
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(+)
ANCILLARY TESTS
Immunohistochemistry
  • 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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