Pseudolymphomas of the Skin


Key Points

  • Cutaneous pseudolymphomas are benign inflammatory skin diseases that mimic malignant lymphomas either clinically, histopathologically, or both.

  • Cutaneous pseudolymphomas should be classified precisely according to specific clinicopathologic entities.

  • Integration of clinical, histopathologic, immunophenotypic and molecular genetic features is crucial for the diagnosis of cutaneous pseudolymphomas.

  • Some non-lymphoid malignant neoplasms may simulate histopathologically the picture of a cutaneous lymphoma.

Introduction

Pseudolymphomas of the skin are benign lymphocytic proliferations that simulate cutaneous malignant lymphomas clinically and/or histopathologically. The term pseudolymphoma does not refer to any particular disease but rather to a heterogeneous group of inflammatory conditions and benign ‘tumors’ of diverse causes. For proper treatment, it is important to identify specific entities. In addition to benign inflammatory skin disorders, some non-lymphoid malignant neoplasms may simulate histopathologically the picture of a cutaneous lymphoma.

History and classification

Cutaneous pseudolymphomas have been known for a long time. In recent years, many inflammatory skin diseases have been added to the group of the cutaneous pseudolymphomas, mainly because of the presence of histopathologic features similar to those observed in malignant lymphomas of the skin. Table 20.1 shows a modern clinicopathologic classification of conditions that are currently viewed as cutaneous pseudolymphomas.

Table 20.1
Classification of Cutaneous Pseudolymphomas
Clinicopathological Entity Simulated Malignant Lymphoma
Actinic reticuloid Mycosis fungoides/Sézary syndrome
Lymphomatoid contact dermatitis
Solitary T-cell pseudolymphoma (superficial type)
Lichenoid (‘lymphomatoid’) keratosis
Lichenoid pigmented purpuric dermatitis (including lichen aureus)
Lichen sclerosus et atrophicus
Vitiligo (inflammatory stages)
Annular lichenoid dermatitis of youth (exact nosology yet unclear)
CD8 + cutaneous infiltrates in HIV-infected patients
Lymphomatoid drug reaction, T-cell type
Pseudolymphomas in tattoos, T-cell type
Pseudolymphomas at sites of vaccination, T-cell type
Mycosis fungoides-like infiltrates in regressing malignant epithelial and melanocytic tumors
Pseudolymphomas in herpes simplex or herpes zoster infections PLEVA, including the febrile ulcero-necrotic variant Cytotoxic NK/T-cell lymphomas or
Lymphomatoid papulosis/cutaneous anaplastic large cell lymphoma
Atypical lymphoid infiltrates (CD30) associated with: orf, milker's nodule, molluscum contagiosum and other infectious disorders Lymphomatoid papulosis/cutaneous anaplastic large cell lymphoma
Persistent arthropod bite reactions (including nodular scabies)
Drug eruptions with clusters of CD30 + lymphocytes
Lupus panniculitis Subcutaneous ‘panniculitis-like’ T-cell lymphoma
Lymphocytoma cutis Follicle center lymphoma
Marginal zone B-cell lymphoma
Diffuse large B-cell lymphoma
Lymphomatoid drug reaction, B-cell type Follicle center lymphoma
Pseudolymphoma after vaccination, B-cell type
Pseudolymphoma in tattoos, B-cell type Marginal zone B-cell lymphoma
Morphea, inflammatory stage
Syphilis (secondary)
‘Acral pseudolymphomatous angiokeratoma’ (small papular pseudolymphoma)
Inflammatory pseudotumor (plasma cell granuloma) Cutaneous plasmacytosis Marginal zone B-cell lymphoma, plasmacytic variant
Lymphocytic infiltration of the skin (Jessner-Kanof) Cutaneous manifestations of B-cell chronic lymphocytic leukemia
Cutaneous extramedullary hematopoiesis Cutaneous manifestations of myeloid leukemia
Reactive angioendotheliomatosis/Intravascular histiocytosis Intravascular diffuse large cell lymphoma
Benign intravascular proliferation of lymphoid blasts

Epidemiology

There are no exact data concerning the incidence, prevalence, and geographic distribution of cutaneous pseudolymphomas. Cutaneous pseudolymphomas induced by the spirochetal microorganism Borrelia burgdorferi (i.e. Borrelia lymphocytoma) commonly arise in regions with endemic Borrelia burgdorferi infection.

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