Clinical features

Sezary syndrome is a rare malignant T-cell lymphoma affecting elderly adults of both sexes, with a predilection for males.

Fig. 3.2 Sezary syndrome. Hyperkeratosis of the palms. Fig. 3.1 Sezary syndrome. Erythroderma. Note enlarged inguinal lymph nodes.

Fig. 3.3 Sezary syndrome. Diffuse hyperpigmentation on the background of erythroderma ('melanoerythroderma'). Note ectropion.

Non-specific skin lesions (eczematous patches) may be present for some time before erythroderma develops. The erythroderma is characterized by intense pruritus and scaling (Fig. 3.1). Common clinical signs are the marked hyperkeratosis of the palms and soles, alopecia and onychodystrophy (Fig. 3.2). Large skin folds (groins, axillae) may be spared. Histopathological analysis of peripheral lymph nodes usually shows evidence of involvement, but differentiation from 'dermatopathic lymphadenopathy' may be very difficult.

As in mycosis fungoides, Sezary syndrome may be associated with follicular mucinosis [34]. Other clinical variants include the presence of diffuse hyperpigmentation as a consequence of melanosis or haemosiderosis (melanoerythro-derma) (Fig. 3.3), and of vesiculobullous lesions.

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