Diagnosis is based on clinical findings, skin biopsy, and laboratory blood tests, and usually requires specialist expertise.
Establishing a diagnosis can be challenging, as the condition can take many different forms in the skin: flat patches, raised plaques, large tumors, and/or marked erythroderma (intense and widespread reddening of the skin).
Early-stage disease is usually managed with skin-directed therapy (topical medications, phototherapy, and localized radiation therapy). If skin disease progresses, or the patient presents with advanced disease, systemic therapies are often necessary (chemotherapy, biologic or immunologic therapy, photopheresis).
Clinical trials may be considered in early and advanced disease. The choice of skin-directed therapy or systemic treatment is usually dependent on both doctor and patient preference, as no one treatment option has been shown to be superior to another.
Prognosis is closely related to the stage at diagnosis. Survival rates in early disease are excellent. However, although treatment may induce remission, patients are expected to relapse after a variable interval and are unlikely to be cured.
Cutaneous T-cell lymphomas (CTCL) make up a heterogeneous group of uncommon disorders characterized by clonal accumulation of T lymphocytes primarily or exclusively in the skin. Mycosis fungoides (MF) and its leukemic variant, Sézary syndrome (SS), are the most common subtypes. MF frequently behaves as an indolent lymphoma with good prognosis for early-stage disease, while SS is considered a more aggressive form of the disease, associated with shortened survival.
History and exam
Key diagnostic factors
- skin patches, plaques, or tumors
Other diagnostic factors
- hypopigmented skin lesions
- unilesional acral site involvement
- constitutional symptoms
- palmar-plantar keratoderma
- leonine facies
- bullous, granulomatous, ichthyosiform, and purpuric lesions
- age >60 years
- male gender
- black ethnicity (mycosis fungoides); white ethnicity (Sézary syndrome)
- exposure to infectious agents
- chromosomal abnormality
- environmental exposure to industrial chemicals, herbicides, pesticides
1st investigations to order
- screen for Sézary cells on blood film
- skin biopsy
- polymerase chain reaction (PCR) for T-cell receptor
- flow cytometry
- basic metabolic panel
- serum LDH
Investigations to consider
- human T-cell lymphotropic virus (HTLV)-I serology
- bone marrow biopsy
- lymph node biopsy
- CT scan
- HIV test
- FDG-PET scan
- Dermatophyte infection
- NCCN clinical practice guidelines in oncology: T-cell lymphomas
- EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome
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