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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