Primary cutaneous CD-8 positive epidermotropic cytotoxic T-cell lymphoma

Definition

A cutaneous lymphoma of CD8-positive cytotoxic T-cells. There is no association with EBV.

Clinical features

Clinical presentation may resemble that of T helper-cell cutaneous T-cell lymphomas, such as mycosis fungoides, pagetoid reticulosis, lymphomatoid granulomatosis, STCL or C-ALCL. Others show a generalised rapidly progressive erythematous eruption with tumours. There may be oral involvement. Visceral dissemination occurs rapidly without lymphadenopathy.

Histopathology

The cutaneous infiltrate is highly epidermotropic, consisting of medium to large pleomorphic lymphocytes, sometimes with a band of immunoblasts in the upper dermis. The epidermis shows spongiosis, vesiculation and necrosis. There may be syringotropism or folliculotropism.

Immunohistochemistry

CD2	variable
CD3	positive
CD4	negative
CD5	variable
CD7	usually positive
CD8	positive
CD16	negative
CD56	negative
CD57	negative
TIA-1	positive
granzyme-B	variable
perforin	variable
TCDd1	positive

Differential diagnosis

see cutaneous T-cell lymphoproliferative disorders

Prognosis

Behavior is aggressive. CD7-negativity is particularly associated with a poor prognosis.

References

Liu, V. and McKee, P.H. Cutaneous T-cell lymphoproliferative disorders: approach for the surgical pathologist: recent advances and clarification of confused issues. Adv Anat Pathol 2002;9:79-100.

This page last revised 27.12.2002.