Mycosis fungoides (cutaneous T-cell lymphoma)

Published on 02/04/2015 by admin

Filed under Internal Medicine

Last modified 22/04/2025

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198 Mycosis fungoides (cutaneous T-cell lymphoma)

Instruction

Look at this patient’s skin.

Salient features

History

• Age (usually fifth and seventh decade)

• Pruritus.

Examination

• Itchy, brownish-red plaques on the hips, buttocks and interscapular region (Fig. 198.1).

image

Fig. 198.1 Patch-stage mycosis fungoides: itchy, brownish-red plaques.

Proceed as follows:

• Tell the examiner that it may also involve the lymph nodes and viscera.

Diagnosis

This patient has itchy brownish-red plaques with lymph node involvement (lesions), indicating mycosis fungoides (aetiology).

Advanced-level questions

How would you confirm the diagnosis?

Skin biopsy to detect Pautrier’s microabscesses, atypical cells in the nests in the epidermis, atypical mononuclear cell infiltrates, large hyperchromic cells with irregular nuclei—the ‘mycosis cells’. The hallmark of mycosis fungoides is the identification of the Sézary–Lautner cells. These are T helper cells (CD4 positive) that characteristically form band-like aggregates within the superficial dermis and invade the dermis as single cells and small clusters (Pautrier’s microabscesses).

What is the prognosis?

Very slow progression of skin lesions with eventual tumour formation and systemic dissemination of lymphoma. In most patients this process takes several weeks.

What is the differential diagnosis?

Psoriasis.

How would you treat this patient?

Treatment is palliative with steroids, chemotherapeutic agents and electron-beam therapy.

What are the stages of cutaneous T-cell lymphoma?

• Stage I: eczematoid or psoriasiform erythematous lesions

• Stage II: infiltrated plaques

• Stage III: nodules (Fig. 198.2), ulcers, tumours

• Stage IV: lymph node involvement with or without systemic dissemination.

image

Fig. 198.2 Mycosis fungoides. Tumour stage III with nodules.

Prognosis is good for early mycosis fungoides (Arch Dermatol 1996;132:1309–13).

Staging is by a tumour/node/metastases/blood system or by the WHO indolent versus aggressive clinical behaviour system (Lancet 2008; 371:945–57).

What is the treatment of mycosis fungoides?

• Various superficial treatments: topical steroids and chemotherapeutic agents (mechlorethamine and carmustine), psoralen plus ultraviolet A (PUVA) and electron-beam radiotherapy improve skin disease, but none has been shown to alter the natural history of the disease.

• Novel approaches being explored include aciclovir, interferons, retinoids, pentostatin, monoclonal antibodies directed to T-cell antigens, extracorporeal photopheresis and interferon-alfa (Lancet 1997;350:32–3).

Have you heard of Sézary syndrome?

It is a rare leukaemic, erythrodermic variant of mycosis fungoides characterized by lymphadenopathy and large mononuclear cells (Sézary cells) in the skin and blood. It is often resistant to traditional treatment and has a poor prognosis. Extracorporeal photopheresis (which consists of oral administration of methoxsalen, exposure of a lymphocyte-enriched blood fraction to ultraviolet A light and reinfusion of the cells into the patient) has recently shown promise.

Further reading

Hwang ST, Janik JE, Jaffe ES, Wilson WH. Mycosis fungoides and Sézary syndrome. Lancet. 2008;371:945-957. (review)

Jean Louis M Alibert, a French dermatologist, first described this condition in 1806.

A Sézary (1880–1956), a French dermatologist.

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