Woman with Nodules on the Abdomen

Evan Farmer, M.D.

Specimen Type:

Skin

History:

The patient is 47-year-old woman with scattered patches and plaques on her abdomen and lower extremities of the least several months duration. She has no systemic symptoms or other abnormal physical findings. The biopsy was from a plaque on her ankle.

Pathologic Features:

The specimen has a superficial perivascular lymphocytic infiltrate with exocytosis into the epidermis tending to aggregate along the dermal-epidermal junction and also forming microabscesses. The majority of lymphocytes are larger than normal with convoluted nuclei. The lymphocytes are positive CD3, CD4 and CD30 with partial loss of CD7.

Differential Diagnosis:

  • Mycosis fungoides
  • Mycosis fungoides-transformed
  • CD30-positive anaplastic large-cell lymphoma
  • Woringer-Kolopp disease

Diagnosis:

Mycosis fungoides-transformed

Mycosis fungoides is a cutaneous T-cell lymphoma and is clinically characterized by the presence of patches and plaques that evolve into cutaneous tumor nodules with subsequent systemic involvement. The disease is histologically characterized by the infiltration of convoluted lymphocytes into the epidermis generally without disturbing the epidermal architecture. The lymphocytes tend to line up along the dermal-epidermal junction and also to aggregate into microabscesses. The lymphocytes tend to have a halo or clear zone around each cell. With time the dermal component takes on a more dermal tumoral aspect and the involvement of the epidermis lessens.

In most cases the lymphocytes express CD4, but there are a few cases with CD8 predominance. The lymphocytes tend to lose the expression of CD7 and other normal lymphocyte markers. The lymphocytes evolve antigen expression over time with some cases expressing CD68 with a granulomatous appearance of the cells and in a few cases develop the expression of CD30 with enlargement of the cells as in our case.

The expression of CD30 on the large lymphocytes within the epidermis has been previously noted and was initially thought to portend a worse prognosis. However, more recent large studies have suggested the worst prognosis was more likely related to the underlying stage of mycosis fungoides or to an early transformation of the lymphocytes.

The presence of CD30-positive lymphocytes indicates the transformation of mycosis fungoides when the underlying histologic and clinical features are consistent with the diagnosis of mycosis fungoides. The proliferation and predominance of large numbers of CD30-positive large lymphoid cells in both the epidermis and especially the dermis in a patient with a small number of nodules raises the possibility of CD30-positive anaplastic large cell lymphoma. The presence of CD30-positive large lymphocytic cells confined to the epidermis in a patient with one or two nodules or plaques raises the possibility of Woringer-Kolopp disease. Clinical pathologic correlation is required to establish the final diagnosis in all of these situations.

References:

  1. Barberio E et al: Transformed mycosis fungoides: clinicopathologic features and outcome. Br J Dermatol 2007;157:284-289.
  2. Wu H, Telang G, Lessin SR, Vonderheid EC: Mycosis fungoides with CD 30-positive cells in the epidermis. Am J Dermatopathol 2000;22:212-216.
  3. Vergier B et al: Transformation of mycosis fungoides: clinicopathologic and prognostic features of 40 cases. Blood 2000;95:2212-2218.