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Integumentary system ORGAN SYSTEMS

Mycosis fungoides (MF)

Introduction

Mature T cell NHL with clinical presentation in the skin (CD4+).

  • M/C cutaneous T-cell lymphoma (CTCL) (constitutes > 50% of all cutaneous lymphomas)
  • Indolent primary cutaneous lymphoma (PCL) (type of extranodal NHL)
  • MF constitutes < 1% of total NHL cases
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The distribution of patients with PCLs | Naeini, F. F., Abtahi-Naeini, B., Pourazizi, M., Sadeghiyan, H., & Najafian, J. (2015). Primary cutaneous lymphomas: A clinical and histological study of 99 cases in Isfahan, Iran. Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences, 20(9), 827–831. doi:10.4103/1735-1995.170595

History

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Jean-Louis-Marc Alibert (1768 – 1837) was a French dermatologist born in Villefranche-de-Rouergue, Aveyron. He was a pioneer of dermatology.

The first clinical description of mycosis fungoides (MF) was made in 1806 by Baron Jean-Louis Alibert, a French physician, who identified a 56-year-old man with skin tumors resembling mushrooms after having a desquamating rash over several months.

In 1975, Lutzner, Edelson, and associates introduced the term cutaneous T-cell lymphoma (CTCL) to describe the spectrum of skin-based lymphomas of T-cell origin, including classic MF and Sézary syndrome (SS).


Classification

Clinical variants:

  • Folliculotropic MF (follicular cell lymphoma)
  • Granulomatous slack skin (rare)
  • Pagetoid reticulosis

Aetiology

  • Human T-cell lymphotropic virus type 1 & 2 (HTLV-1/2) (associated with Adult T cell leukaemia/lymphoma)

Pathophysiology

Tumour cells originate from memory T cells or skin-homing CD4+ T cells expressing cutaneous lymphocyte antigen (CLA) and chemokine receptors CCR4 and CCR7.

Patch → Plaque → Tumour → Erythroderma: Sézary syndrome (SS)


Clinical features

Truncal scaling, red-brown patches, plaques and fungating nodules seen

Patch stage:

  • Earliest lesion: Erythematous/brownish scaly patch (may show slight atrophy)
  • Plaque parapsoriasis: Single/multiple lesions of variable diameters and locations develop, often in covered areas and particularly in the gluteal region and on the proximal thighs.
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Mycosis fungoides “patches” stage. | Dove Press. (2020) [Full text] Management of mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTC | CMAR. Retrieved February 03, 2020, from https://www.dovepress.com/management-of-mycosis-fungoides-type-cutaneous-t-cell-lymphoma-mf-ctcl-peer-reviewed-fulltext-article-CMAR

Plaque stage:

  • Multiple larger lesions with evident infiltration
  • Annular/horseshoe-shaped lesions with an infiltrated base, raised, well-defined edges and asymmetrical distribution.
  • May also affect the face and the scalp
cmar-138661_F002
Mycosis fungoides “plaques” stage | Dove Press. (2020) [Full text] Management of mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTC | CMAR. Retrieved February 03, 2020, from https://www.dovepress.com/management-of-mycosis-fungoides-type-cutaneous-t-cell-lymphoma-mf-ctcl-peer-reviewed-fulltext-article-CMAR

Tumour stage:

  • Erythematous-purplish papules or nodules of larger diameter seen

Complication

Sézary syndrome (SS):

  • MF evolves into a systemic lymphoma with visceral and nodal spread.

Diagnosis

Excisional skin biopsy:

  • Pautrier microabscesses: tumour cells invade epidermis as single cells and small clusters
  • Epidermotropism
2-Figure2-1
A, B, Pautrier microabscesses containing atypical lymphocytes and apoptotic cells (arrows) [hematoxylin and eosin, original magnification (OM): 3200]. For comparison, the inset in (B) (OM: 340) shows 2 typical microabscesses of MF without apoptotic cells. C, Apoptotic debris in the context of a dense infiltrate of atypical lymphocytes (OM: 3100). | Kim, N.H., Torchia, D., Miteva, M., Rongioletti, F., & Romanelli, P.L. (2011). Prominent apoptosis in pautrier microabscesses: a distinctive finding in adult T-cell leukemia/lymphoma? The American Journal of dermatopathology, 33 5, 530-1 .

Differential diagnosis:

  • Psoriasis
  • Chronic eczema
  • Atopic dermatitis
  • Leprosy/lichenoid pityriasis

Tumor-node-metastasis-blood (TNMB) staging:


Management

Skin-directed therapy:

  • Topical/systemic steroids
  • Topical nitrogen mustard
  • Phototherapy (UVB and PUVA)
  • Total skin electron beam therapy (TSEBT)

Radiation therapy:

  • For local control of skin and nodal disease.

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