Sezary Syndrome

Key Points
Condition is form of T-cell lymphoma causing the skin of the entire body to become bright red, thickened, dry or scaly and usually very itchy.
Condition has a poor survival rate.

Sézary syndrome is the name given when T-cell lymphoma — or tumors of the lymph nodes and lymphatic system occurring in the skin with no evidence of disease anywhere — affects the skin of the entire body. It is also known as the Red Man syndrome because the skin is bright red. The skin is also thickened, dry or scaly and usually very itchy. Examination usually reveals the presence of Sézary cells in skin, lymph nodes, and peripheral blood. The prognosis of Sézary syndrome is generally poor with a median survival between 2 and 4 years. Most patients die of infections that are due to immunosuppression.

Differential Diagnosis (Other conditions with similar appearance)
Atopic Dermatitis
Contact Dermatitis
Lichen Planus
Tinea Corporis

Diagnosis
Key Points
A dermopathologist should do the skin biopsy, as there are microscopic changes that need to be determined.
Early T-cell lymphoma can be difficult to differentiate from other skin conditions.
An elevated white cell count is characteristic of Sezary syndrome.

The diagnosis should be made by a dermatopathologist as there are characteristic microscopic changes seen on skin biopsy. The diagnosis is often delayed for months or years and may require several biopsies, as early cutaneous T-cell lymphoma can be difficult to tell apart from other skin conditions, particularly eczema. Enlarged lymph nodes may also be biopsied. Cutaneous T-cell lymphoma can cause harmless swelling, or result in malignant growth in the lymph nodes. An elevated white cell count is characteristic of Sézary syndrome. Patients with advanced cutaneous T-cell lymphoma may have CT or MRI scans to determine whether the disease affects internal organs.

Treatment
Key Points
Treatment depends on the stage and available drugs and equipment.

Treatment of individual patients varies, and depends on the stage and available drugs and equipment. Such treatments would include topical steroids, UVB phottherapy, PUVA photochemotherapy, topical nitrogen mustard, bexarotene gel, chemotherapy, localized radiotherapy, electron beam radiotherapy, interferons, oral retinoids, and photopheresis.