6-14: Cutaneous T-Cell Lymphoma (Mycosis Fungoides)

ESSENTIALS OF DIAGNOSIS

GENERAL CONSIDERATIONS

Mycosis fungoides is a cutaneous T-cell lymphoma that begins on the skin and may remain there for years or decades. It may progress to systemic disease, including Sézary syndrome (erythroderma with circulating malignant T cells).

CLINICAL FINDINGS

A. Symptoms and Signs

Localized or generalized erythematous patches or scaly plaques are present usually on the trunk. Plaques are almost always over 5 cm in diameter. Pruritus is a frequent complaint and can be severe. IL-31 may mediate the pruritus of Sézary syndrome. The lesions often begin as nondescript patches, and patients may have skin lesions for more than a decade before the diagnosis is confirmed. Follicular involvement with hair loss is characteristic of mycosis fungoides, and its presence should raise the suspicion of mycosis fungoides for any pruritic eruption. In more advanced cases, tumors appear. Local or diffuse lymphadenopathy may be due to benign expansion (dermatopathic lymphadenopathy) or involvement with mycosis fungoides.

B. Laboratory Findings

Diagnosis is based on skin biopsy though numerous biopsies may be required before the diagnosis is confirmed. In more advanced disease, circulating malignant T cells (Sézary cells) can be detected in the blood (T-cell gene rearrangement test). Eosinophilia may be present.

DIFFERENTIAL DIAGNOSIS

Mycosis fungoides may be confused with psoriasis, drug eruption, photoallergy, eczematous dermatitis, syphilis, or tinea corporis. Histologic examination can distinguish these conditions.

TREATMENT

The treatment of mycosis fungoides is complex. Early and aggressive treatment has not been proven to cure or prevent disease progression. Skin-directed therapies, including topical corticosteroids, topical mechlorethamine, bexarotene gel, and UV phototherapy, are used initially. If the disease progresses, PUVA plus retinoids, PUVA plus interferon, extracorporeal photopheresis, bexarotene, histone deacetylase inhibitors (romidepsin or vorinostat), targeted immunomodulators (brentuximab, mogamulizumab), and total skin electron beam treatment are used.

PROGNOSIS

Mycosis fungoides is usually slowly progressive (over decades). Prognosis is better with patch or plaque stage disease and worse with erythroderma, tumors, and lymphadenopathy. Survival is not reduced in patients with limited patch disease. Elderly patients with limited patch and plaque stage disease commonly die of other causes. Overly aggressive treatment may lead to complications and premature demise.