Last reviewed: November 2017
Last updated: June  2017

Summary

Definition

History and exam

Key diagnostic factors

  • skin patches, plaques, or tumors
  • poikiloderma
  • erythroderma

Other diagnostic factors

  • pruritus
  • hypopigmented skin lesions
  • unilesional acral site involvement
  • lymphadenopathy
  • constitutional symptoms
  • palmar-plantar keratoderma
  • alopecia
  • leonine facies
  • onychodystrophy
  • hepatomegaly
  • ectropion
  • bullous, granulomatous, ichthyosiform, and purpuric lesions

Risk factors

  • age >60 years
  • male gender
  • black ethnicity (mycosis fungoides); white ethnicity (Sézary syndrome)
  • exposure to infectious agents
  • chromosomal abnormality
  • environmental exposure to industrial chemicals, herbicides, pesticides

Diagnostic investigations

1st investigations to order

  • CBC
  • screen for Sézary cells on blood film
  • skin biopsy
  • PCR for T-cell receptor
  • flow cytometry
  • basic metabolic panel
  • LFTs
  • serum LDH
Full details

Investigations to consider

  • human T-cell lymphotropic virus (HTLV)-I serology
  • bone marrow biopsy
  • lymph node biopsy
  • CT scan
  • HIV test
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Professor of Targeted Therapy and Oncology

University of Manchester

Manchester Academic Health Sciences Centre

Manchester

UK

Disclosures

TMI declares that he has no competing interests.

Consultant in Clinical Oncology

Christie NHS Foundation Trust

Manchester

UK

Disclosures

RC declares that he has no competing interests.

Consultant Dermatologist

Tameside Hospital Integrated Care Organisation NHS Trust

Manchester

UK

Disclosures

EP declares she has no competing interests.

Peer reviewers VIEW ALL

Assistant Professor

Department of Radiation Oncology

Duke University School of Medicine

Durham

NC

Disclosures

CK declares that he has no competing interests.

Professor and Head of Dermatology

Department of Medicine

New Jersey Medical School

Newark

NJ

Disclosures

RAS declares that he has no competing interests.

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