Contents

SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferences

Information for Patients

View all Images (15)

Cutaneous T-cell lymphoma - Nail and Distal Digit
See also in: Overview
Other Resources UpToDate PubMed

Cutaneous T-cell lymphoma - Nail and Distal Digit

See also in: Overview
Contributors: Shari Lipner MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Cutaneous T-cell lymphomas (CTCLs) are a heterogeneous group of neoplasms that vary considerably in their clinical presentation, histology, immunophenotype, genetics, and prognosis. Mycosis fungoides (MF) and its variants (folliculotropic mycosis fungoides, pagetoid reticulosis, and granulomatous slack skin), Sézary syndrome, lymphomatoid papulosis, and cutaneous anaplastic large cell lymphoma make up 90% of all CTCL cases. CTCLs are seen most often in the elderly, but they can occur in patients of all ages. The definitive diagnosis of CTCL may require large or multiple biopsies as well as specialized testing of the biopsy specimens. Typing of the CTCL and staging are important to determine the extent of disease and treatment strategy.

Nail changes are common in patients with CTCL, especially those with Sézary syndrome, and typically affect more than one nail. In many cases, changes affect all 20 nails. At times, the nail changes are due to treatments of CTCL and not to the disease itself. For example, Beau lines have been observed with chemotherapy, and melanonychia was seen with psoralen plus ultraviolet A (PUVA) therapy.

In a retrospective study of 83 patients with Sézary syndrome, 36 patients (43.4%) were found to have nail changes. Nail thickening, nonspecific nail dystrophy, nail plate yellowing, and subungual hyperkeratosis were among the most common nail findings (42%-58%). Other nail changes were onycholysis, nail plate ridging, and splinter hemorrhages, seen in 17%-19% of patients. Beau lines and onychomadesis were seen in 8% of patients. In another study, 52.9% (45 of 85) of patients with Sézary syndrome who had keratoderma had positive potassium hydroxide (KOH) tests under microscopy on skin scrapings. Therefore, some of these nail changes may be attributable to onychomycosis. Paronychia was frequently reported in another study of 19 patients with Sézary syndrome (63.2%).

Small case series and case reports have described nail discoloration and thickening, onycholysis, crumbling, subungual hyperkeratosis, anonychia, onychomadesis, and splinter hemorrhages in patients with MF. In a retrospective study of 60 patients with biopsy-proven MF, 18 (30%) had nail changes. The most common nail findings were longitudinal ridging, nail plate thickening, nail fragility, and leukonychia.

Codes

ICD10CM:
C84.A0 – Cutaneous T-cell lymphoma, unspecified, unspecified site

SNOMEDCT:
400122007 – Primary cutaneous T-cell lymphoma

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Differential diagnosis of the nail changes of CTCL:
  • Psoriasis – Nail changes include the oil-drop sign, onycholysis, subungual hyperkeratosis, splinter hemorrhages, and nail pits.
  • Atopic dermatitis – Eczema involving the nail folds may lead to nail ridging, coarse pitting, and other dystrophic changes secondary to inflammation of the underlying matrix.
  • Onychomycosis
  • Pityriasis rubra pilaris – Follicular-based scaly papules with an orange hue become confluent to form plaques. Palms have an orange-hued keratoderma. Nails display subungual hyperkeratosis and thickening.
  • Reactive arthritis – Keratoderma blenorrhagicum and nail involvement are common findings. Paronychia, onycholysis, ridging, splitting, elkonyxis (loss of nail plate substance above the lunula only), a brown-red discoloration, and subungual hyperkeratosis may be seen.

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

Subscription Required

References

Subscription Required

Last Reviewed:07/15/2020
Last Updated:02/14/2022
Copyright © 2023 VisualDx®. All rights reserved.
Cutaneous T-cell lymphoma - Nail and Distal Digit
See also in: Overview
A medical illustration showing key findings of Cutaneous T-cell lymphoma (Mycosis Fungoides) : Hyperpigmented patch, Lymphadenopathy, Tumor, Pruritus
Clinical image of Cutaneous T-cell lymphoma - imageId=70243. Click to open in gallery.  caption: 'Patch stage mycosis fungoides, showing thin scaly erythematous and brown plaques on the lower abdomen.'
Patch stage mycosis fungoides, showing thin scaly erythematous and brown plaques on the lower abdomen.
Copyright © 2023 VisualDx®. All rights reserved.