Sézary's disease

Sezary syndrome, named after the French dermatologist Albert Sézary (1880-1956), is a cutaneous T -cell lymphoma with the symptoms: large-scale skin redness ( erythroderma ), severe itching (pruritus ), lymph node enlargement, often hair loss ( alopecia) the entire body hair, an excessive Hautverhornung (hyperkeratosis ) and nail malformations ( onychodystrophy ).

The Sezary syndrome is characterized by circulating in the blood and in the skin biopsy found on such atypical ( degenerate ) T lymphocytes ( Lutzner cells).

Most important differential diagnoses of Sezary syndrome is the mycosis fungoides, the most common subtype of cutaneous T -cell lymphomas. Both diseases are treated with PUVA, partly in combination with retinoids, the Sézary syndrome have a worse prognosis than the mycosis fungoides with about 50 % cure rate. When Sezary syndrome also extracorporeal photopheresis is effective. In advanced stages, therapeutic trials with interferon or chemotherapeutic agents can be undertaken.

Other differential diagnoses include atopic skin diseases eczema and psoriasis.