Abstract

CD30 expression is most commonly associated with primary cutaneous CD30-positive lymphoproliferative disorders including cutaneous-anaplastic large cell lymphoma and lymphomatoid papulosis (LyP) types A-E. The reports of CD30-positive mycosis fungoides (MF) include non-transformed MF, transformed CD30-positive MF, MF associated with LyP, or MF associated with anaplastic large cell lymphoma. Rarely, folliculotropic mycosis fungoides presents with a CD30-positive clonal population, most often in association with large cell transformation. Even sparser in the literature is CD30-positive primary folliculotropic cutaneous t-cell lymphoma presenting as alopecia. We report the case of a 74-year-old female who presented with generalized pruritus and progressive non-scarring alopecia of her body hair and scalp for 8 months. Biopsies of the plaques of alopecia on the scalp revealed epidermotropic and folliculotropic atypical lymphocytes with follicular mucinosis. The atypical lymphocytes stained positive for CD3, CD4, CD30, CD45RB and did not stain with antibodies against CD8 and CD45RO. Blood flow cytometry subsequently demonstrated an atypical clonal population with positive reactivity to: CD2, CD3, CD4, CD5, and CD30 (23.5%), and negativity for CD7. These findings confirmed CD30 positive primary folliculotropic cutaneous t-cell lymphoma in association with CD30-positive Sezary syndrome. It is unclear whether blood involvement proceeded or followed skin involvement. The expression of CD30 positivity, as in this case report, may confer a worse prognosis in patients with advanced stage mycosis fungoides or Sezary syndrome. Increased attention to the presence of CD30-positive lymphoproliferative disorders and the potential overlap between presentations of mycosis fungoides and lymphomatoid papulosis is warranted.

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