Mycosis Fungoides / Sezary Syndrome

Snapshot

A 55-year-old man complains of a rash he has had for many months. It occasionally itches. While he has tried some topical over-the-counter steroids, he has not seen his dermatologist yet since the symptoms were mild. On physical exam, his legs are covered with multiple well-demarcated red papules and plaques with some telangiectasias. A skin biopsy is obtained. A week later, the biopsy comes back with Pautrier microabsecesses, lymphocytic infiltrate, and cells with cerebriform nuclei.

Introduction

Both are variants of cutaneous T-cell lymphoma (CTCL)
- helper CD4+ T-cell lymphoma of the skin
Mycosis fungoides (50-70% of CTCLs)
- skin involvement only
Sézary syndrome (1-3% of CTCLs)
- leukemic form of CTCL
- CTCL + malignant T-cells in the blood
Epidemiology
- older patients (median age 55-60)
- more common in male patients
more common in black patients

Presentation

Evaluation

Physical exam to assess for lymphadenopathy and organomegaly
Diagnosis usually made by clinical exam and history
Diagnosis confirmed with skin biopsy
- lymphocytic infiltrate with Pautrier microabscesses within thickened epidermis.
- “buttock cells”: atypical lymphocytes with cerebriform nuclei (Sézary cells)
Labs
complete blood count with peripheral blood smear to look for Sézary cells (atypical T-cells)

Differential Diagnosis

Treatment

Pharmacologic options
- topical steroids
- topical chemotherapies
- radiation therapy
interferon

Prognosis, Prevention, and Complications

Prognosis
- stage-dependent
- mycosis fungoides typically indolent
  - slow-growing
- Sézary syndrome typically aggressive
  - can be fatal
Complications
- intense pruritus
- secondary cutaneous bacterial or viral infection