Lymphomatoid papulosis-Low magnification

LEGEND

Lymphomatoid papulosis is a primary cutaneous low-grade T-cell lymphoma composed of large CD30+ cells (i.e., more than 4× the size of a normal lymphocyte).

There are several histologic subtypes, from type A to F. Type A accounts for >75% of cases. The subtypes are histopathologically relevant but have no clinical impact.

Type A typically has an inverted triangle (“wedge shape”) appearance.

Mixed inflammation with occasional presence of normal lymphocytes, neutrophils, and eosinophils.

  • Dermal nodular infiltrate of large atypical CD30+ T lymphocytes (i.e., >4× the size of a normal lymphocyte), with occasional epidermotropism.

  • Epidermal necrosis or ulceration may sometimes be observed, with epidermal hyperplasia (especially in type E, shown here).

Lymphomatoid papulosis-High magnification

LEGEND

Atypical mitoses may be observed

  • The epidermis is often reactive: presence of acanthosis and spongiosis, as well as papillary dermal edema (unlike in classic MF).

  • Epidermotropism

Lymphomatoid papulosis-Type C-Low magnification

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  • Diffuse proliferation in the dermis of large atypical CD30+ T lymphocytes

Lymphomatoid papulosis-Type C-High magnification

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  • Presence of large atypical CD30+ T lymphocytes, i.e., >4× the size of a normal lymphocyte

Lymphomatoid papulosis-Type C

CD3 may be positive or negative

In this case, CD3 expression is positive.

Lymphomatoid papulosis-Type C

CD4 positive

Lymphomatoid papulosis-Type C

CD30 positive

Lymphomatoid papulosis-Type C

ALK1 negative (marker of systemic anaplastic large cell lymphoma)

Lymphomatoid papulosis-Type C

EMA negative (marker for systemic anaplastic large cell lymphoma)

Lymphomatoid papulosis-Type E

LEGEND

Type E is characterized by angioinvasion, angiodestruction, and vascular occlusion, sometimes with presence of thrombi.

Epidermal ulceration and necrosis is extensive

Large CD30+ T lymphocytes are seen in the infiltrate.

  • Infiltrate of atypical lymphocytes

Lymphomatoid papulosis-Type E

CD3 may be positive or negative.

In this case, CD3 expression is positive.

Lymphomatoid papulosis-Type E

CD20 is negative in the dermal infiltrate.

A slight reactive positivity is seen at the site of ulceration.

Lymphomatoid papulosis-Type E

CD30 expression is positive (large cells) in the dermal infiltrate around the ulceration.

CD30 is typically negative in type B.

Lymphomatoid papulosis-Type E

ALK1 negative (marker for systemic anaplastic large cell lymphoma)