Case Five – May 2017

  • Patient Information
    • 51 year old female presented with numerous rapidly-progressive crusted and ulcerated plaques and nodules on the trunk and extremities
  • Clinical Diagnosis
    • Infection
    • Drug eruption
    • Lymphomatoid papulosis
    • Lymphoma
  • Histology

    • Sections show a band-like and epidermotropic lymphoid infiltrate with accompanying ulceration and hemorrhage.

    • The epidermotropic lymphocytes are atypical with enlarged, hyperchromatic nuclei and irregular nuclear contours. Associated necrotic keratinocytes are seen.

    Immunohistochemistry:

     

    • The atypical lymphoid cells are positive for CD3, CD5, CD8, BF1, TIA-1 and granzyme B.
    • The atypical lymphoid cells are negative for CD4 and TCR gamma M1.
  • Final Diagnosis

    Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (CD8+ AECTCL)

    • This is a very rare lymphoma of CD8+ cytotoxic T-cells that express TCR alpha/beta
    • CD8+ AECTCL may have been described previously as generalized pagetoid reticulosis (Kettron-Goodman disease)
    • Most patients are in their fifth to seventh decade
    • Typically presents as disseminated hyperkeratotic patches and plaques or hemorrhagic plaques and nodules that rapidly spread and often ulcerate
    • Histologic features include a band-like to diffuse infiltrate of medium to large atypical lymphocytes with prominent epidermotropism and frequent involvement of the adnexal structures
    • Necrotic keratinocytes, spongiosis, and ulceration commonly accompany the epidermtropic lymphocytes
    • Immunohistochemical stains show the atypical lymphocytes are positive for CD3, CD8, BF1, CD45RA, TIA-1, granzyme B, perforin and negative for CD4, CD30, CD45RO, CD56
    • Variable loss of CD2 and CD5 may also be seen
    • The main histologic differential diagnosis includes CD8+ mycosis fungoides, which typically lacks necrotic keratinocytes and ulceration
    • Pagetoid reticulosis can mimic CD8+ AECTCL histopathologically, but pagetoid reticulosis is defined as a localized lesion with an indolent clinical course
    • Lymphomatoid papulosis type D histologically mimics CD8+ AECTCL but is CD30-positive and spontaneously regresses clinically
    • Extracutaneous involvement of the oral mucosa, testis, lung, and central nervous system have been described but lymph nodes are often spared
    • CD8+ AECTCL has an aggressive clinical course with a median survival of 32 months
  • Summary
    • Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma is an extremely rare, rapidly-progressive lymphoma of alpha/beta T-cells
    • The atypical lymphocytes are positive for CD3, CD8, BF1, CD45RA, TIA-1, granzyme B, perforin and negative for CD4, CD5, CD30, CD45RO, and CD56
    • Extracutaneous involvement of the oral mucosa, testis, lung, and central nervous system can occur
    • CD8+ AECTCL has an aggressive clinical course with a median survival of 32 months