Lymphomatoid Papulosis
C. Cameron Yin, MD, PhD
Key Facts
Terminology
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Chronic, self-healing and recurrent erythematous papules/nodules on skin of trunk and extremities
Clinical Issues
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Usually remains confined to skin
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Excellent prognosis
Microscopic Pathology
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4 histologic types represent a spectrum of disease
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Type A is most common
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Scattered large atypical Reed-Sternberg-like cells
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Numerous inflammatory cells including small lymphocytes, histiocytes, granulocytes
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Type B is uncommon (< 10%)
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Epidermotropism and band-like dermal infiltrate
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Lymphoid cells with cerebriform nuclei
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Type C
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Clusters or sheets of large atypical lymphoid cells with relatively few admixed inflammatory cells
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Type D
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Epidermotropism; CD8(+)
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Ancillary Tests
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Large atypical cells are CD30(+)
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Small lymphocytes are T cells: Usually CD7(-)
Top Differential Diagnoses
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Primary cutaneous ALCL
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Systemic ALCL with cutaneous involvement
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Classical Hodgkin lymphoma
Reporting Considerations
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In many cases, LyP cannot be distinguished from C-ALCL without clinical information/follow-up
TERMINOLOGY
Abbreviations
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Lymphomatoid papulosis (LyP)
Synonyms
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Primary cutaneous CD30(+) T-cell lymphoproliferative disorder
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This term also includes cutaneous anaplastic large cell lymphoma (C-ALCL)
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Definitions
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Chronic, self-healing and recurrent skin lesions characterized by erythematous papules/nodules on trunk and extremities
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Composed of large atypical cells in marked inflammatory background
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Initially described by Macaulay as “a continuing self-healing eruption, clinically benign, histologically malignant”
ETIOLOGY/PATHOGENESIS
Unknown
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Suggested factors
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Viral infection, reduced immunosurveillance
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Chronic antigenic stimulation, direct oncogenic effect of immunosuppressive drugs
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Outbreaks may be triggered by stress or illness
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TNFR-associated factor-1 and cutaneous lymphocyte antigen (E-selectin ligand) are highly expressed in LyP
CLINICAL ISSUES
Epidemiology
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Age
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Median: 45 years (wide age range, including children)
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Gender
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Male to female ratio = 2-3:1
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Site
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Trunk and extremities most common
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Genital and oral mucosa can be rarely involved
Presentation
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Papular, papulonodular, or nodular skin lesions at different stages of development
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Clusters or disseminated; ± ulceration
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Individual skin lesions spontaneously regress within 3-12 weeks
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After resolution, superficial scars can remain; hypo-or hyperpigmented
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Waxing and waning clinical course; can persist for decades
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LyP usually remains confined to skin
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Can disseminate to regional lymph nodes
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Very rarely disseminates elsewhere
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Treatment
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No specific therapy for most patients; follow-up with attention to skin lesion changes or development of lymphadenopathy
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Therapy options include
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Surgical removal ± irradiation or low-dose methotrexate for skin-restricted disease
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Multiagent chemotherapy for extracutaneous lesions
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Prognosis
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Excellent
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10-year disease-specific survival of ˜ 100%
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Spontaneous regression in > 40% of patients
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10-20% of patients develop a 2nd lymphoma
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Mycosis fungoides (MF), C-ALCL, or classical Hodgkin lymphoma
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LyP patients have increased risk for nonlymphoid cancers
MICROSCOPIC PATHOLOGY
Histologic Features
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Typically wedge-shaped lesion involving dermis
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Epidermis is usually sparsely infiltrated and often ulcerated
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4 histologic types have been recognized, which represent a spectrum of disease
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Arbitrarily designated as A, B, C, and D
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Type A is most common
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Scattered large atypical Reed-Sternberg-like cells
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Numerous inflammatory cells including small lymphocytes, histiocytes, neutrophils, and eosinophils
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Type B is uncommon (< 10%)
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Simulates MF with epidermotropism and band-like dermal infiltrate
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Composed of small to medium-sized lymphoid cells with cerebriform nuclei
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Cannot be distinguished from MF by histology or immunophenotyping alone
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Unlike MF, type B LyP usually regresses spontaneously
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Type C
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Large clusters or sheets of large atypical lymphoid cells with relatively few admixed inflammatory cells
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Cannot be distinguished from C-ALCL by histology or immunophenotyping alone
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Type C LyP is smaller (usually < 10 mm) than C-ALCL and spontaneously regresses over time
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Type D has been recently described
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Characterized by marked epidermotropism and CD8(+)
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ANCILLARY TESTS
Immunohistochemistry
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Types A and C
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Large atypical cells are CD30(+), ALK(-)
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Small lymphocytes are T cells
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CD2(+), CD3(+), CD5(+), CD7 often (-); CD4(+), CD8(-)
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Frequent expression of cytotoxic proteins: TIA-1, granzyme B, &/or perforin
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Type B: Small cells with cerebriform nuclei are CD3(+), CD4(+), CD8(-), CD30(-)
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Occasionally LyP has CD8(+) immunophenotype
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More common in children
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Type D: Atypical lymphocytes are CD30(+), CD3(+), CD4(-), CD8(+)

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