Track
Case ReportsAbstract
Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell
lymphoma (CD8+ AECTCL) is a newly recognized, rare entity with a typically dismal
prognosis. Its diagnosis is challenging due to significant clinical and histologic overlap
with more indolent conditions like CD8+ Mycosis Fungoides (CD8+ MF), and
Lymphomatoid Papulosis (LyP), a distinction with prognostic implications.
To investigate this, we performed a retrospective search of our institution's database
over 25 years (1999-2024), identifying eight cases of CD8+ T-cell lymphoma with
epidermotropism. The initial clinical impressions varied widely: six cases mimicked
inflammatory dermatoses (e.g., eczema, psoriasis, tinea corporis, or arthropod bite
reaction), while only two were initially suspected to be CTCL.
Our cohort consisted of five males and three females, with a wide age range (15-77
years) and a predilection for the lower extremities.
Histologically, all cases were CD8-positive with significant epidermotropism.
Immunohistochemical analysis revealed variable expression of CD30, cytotoxic markers
(Granzyme B/TIA-1), and CD56, with frequent aberrant loss of CD7. Molecular data was
available for only one case, which showed TCR clonality; further molecular exploration
of these cases is planned.
Clinical follow-up, available for four patients (ranging from 12 to 132 months), revealed
a crucial finding: all patients had an indolent clinical course and were managed
conservatively. This favorable outcome, despite histologic features that can mimic the
aggressive CD8+ AECTCL, underscores the limitations of relying on biopsy findings
alone and highlights the essential role of clinical correlation in diagnosing these
challenging lymphomas.