Case Report
Three cases of lymphomatoid papulosis with a CD56+ immunophenotype

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We report 3 cases of lymphomatoid papulosis (LyP) with a CD56+, cytotoxic immunophenotype. All 3 patients presented with clinical histories typical of LyP, with one patient having associated mycosis fungoides. Histologically, two cases were type A LyP and one was type B. All 3 cases demonstrated a T-cell receptor clone in lesional skin without evidence of blood involvement. The atypical lymphocytes in each of the 3 cases expressed cytotoxic granules (T-cell intracellular antigen-1+ and granzyme B+) and were CD8+ and CD56+. Expression of CD56 is associated with a poor prognosis in subcutaneous panniculitis-like T-cell lymphoma and blastic natural killer cell lymphoma. However, the two cases of CD56+ LyP previously reported and the 3 cases in this series all appear to be pursuing an indolent course with no evidence of systemic disease.

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Case 1

A 29-year-old Caucasian man presented in 2002 with a 4-year history of recurring erythematous, crusted papules and nodules occurring on his arms and legs. The lesions lasted 2 to 3 months and healed with postinflammatory hyperpigmentation. He had no hepatosplenomegaly or lymphadenopathy. The disease pursued an indolent course with no treatment and the patient was subsequently lost to follow-up.

A skin biopsy specimen of a papule showed an epidermotropic infiltrate of medium atypical lymphocytes

Discussion

Only two cases of LyP with a CD56+ immunophenotype have been reported in the literature.3, 4 The initial case, reported by Bekkenk et al,3 described a patient with a 20-year history of recurrent self-healing skin lesions together with a CD30+ lymphoproliferative disease histologically, consistent with a diagnosis of LyP. The immunohistochemical findings were CD30+ and CD56+, but CD2, CD3, CD4, CD8, and TIA-1. The disease had already pursued an indolent course for 20 years and was

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Funding sources: None.

Conflicts of interest: None identified.

Reprint requests: Robin Russell-Jones, MA, FRCP, FRCPath, St John's Institute of Dermatology, St Thomas' Hospital, Lambeth Palace Rd, London SE1 7EH, United Kingdom.

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