Non-Classic Signs of S ézary Syndrome: A Review

AbstractThe majority of patients with S ézary syndrome (SS) present with classic symptoms of erythroderma, lymphadenopathy, and pruritus. However, there have been numerous reports of patients with SS who have non-classic signs. In this review, we report the less common clinical presentations of SS and discuss their relevant treatments. O ur search included all literature on SS since 2008, the year the World Health Organization (WHO) incorporated the diagnostic criteria for SS into theWHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. We reviewed 896 articles and identified 505 patients with non-classic presentations of SS. Of these 505 patients, the most common non-classic signs of SS were keratoderma, onychodystrophy, alopecia, leonine facies, and ectropion. Given the aggressive and highly symptomatic nature of SS, it is imperative that clinicians recognize the less common signs of the disease to prevent delays in diagnosis and treatment. To our knowledge, this is the first review of the clinical variations of SS with a focus on non-classic signs and symptoms.

Source: American Journal of Clinical Dermatology - January 16, 2020 Category: Dermatology Source Type: research