How is erythroderma characterized in Sézary syndrome cutaneous T-cell lymphoma (CTCL)?

Updated: Aug 15, 2018
  • Author: Lauren C Pinter-Brown, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Sézary syndrome is characterized by erythroderma, which is often associated with marked pruritus and exfoliation, edema, and lichenification. Lymphadenopathy, onychodystrophy, and palmoplantar hyperkeratosis are also commonly associated. In addition, patients experience thickening of the facial skin folds (leonine facies) and ectropion of the eyelids. Sun exposure may be painful, as well as pruritic. (See the image below.)

Erythroderma of Sézary syndrome. Erythroderma of Sézary syndrome.

A few patients with Sézary syndrome have patchy, total-scalp, or universal alopecia. Follicular mycosis fungoides may present with alopecia; total-body hair loss may be evident in some patients with generalized erythroderma and Sézary syndrome. [65] The alopecia may also appear clinically identical to alopecia areata. However, skin biopsy specimens may reveal atypical T lymphocytes within the follicular epithelium or epidermis, sometimes with follicular mucinosis.


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