Abstract
Key Clinical MessageSézary syndrome is an extremely rare form of cutaneous T‐cell lymphoma. It presents suddenly and is associated with a poor prognosis. Clinical recognition is crucial for the diagnostic process and initiation of appropriate treatment. Plantar keratoderma is usually pathognomonic for Sézary syndrome and clinicians should be alerted to its presence.
Highlights
Plantar keratoderma is usually pathognomonic for Sezary syndrome and clinicians should be alerted to its presence
A 65-year-old woman presented with a 4-year history of progressive refractory diffuse pruritus
After being treated unsuccessfully with PUVA for 31 sessions, she was eventually diagnosed with cutaneous T-cell lymphoma (CTCL) stage IVA2 (Sezary syndrome) on the basis of severe worsening pruritus, erythroderma, palmoplantar keratoderma with skin ulcerations causing cellulitis (Fig. 1A), left eye ectropion, lymphanedopathy, positive skin biopsy and left inguinal lymph node biopsy, raised lactate dehydrogenase, Sezary cells over 1000/lL with positive clonality, CD4/CD8 ratio: 12.5, and CT chest, abdomen and pelvis showing inguinal and axillary lymphadenopathy with no visceral involvement
Summary
University College London Hospitals, 250 Euston Road, London NW1 2PG, UK. Tel: +44 7960 340489; Fax: +44 (0) 203 447 9217 E-mail: constantinos.frangos.09@ucl.ac.uk Funding Information No sources of funding were declared for this study. Received: 11 July 2017; Accepted: 5 August 2017
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.