Signs and Symptoms of Cutaneous T Cell Lymphoma (CTCL)
Clinically, as well as pathologically, cutaneous T cell lymphoma (CTCL) has four stages of progression: patch stage, plaque stage, tumor stage, and erythroderma stage. The disease can manifest at any stage and does not necessarily go through an orderly progression.
In addition, erythroderma (red skin) can develop, as well as a systemic blood disorder known as Sezary syndrome, which involves the migration of malignant T cells throughout the body.
The patch stage is characterized by flat, pink areas of skin, inflammation, and, sometimes, itching. The patches may remain for years and can resolve spontaneously. They can vary in size and are usually scaly. These nonspecific patches can make diagnosis difficult, clinically and pathologically, because they look like several other benign skin disorders.
In the plaque stage, the cells multiply and grow, and the lesions thicken, become elevated, and show textural changes. Plaques may be annular (forming a ring); pink, white, or brown; and are often scaly. As the plaques thicken, the diagnosis becomes more obvious.
In the tumor stage, multiple reddish brown or purplish red nodules develop. The face and body folds are usually the sites of tumor development. The tumor may ulcerate and become infected. Spontaneous resolution of tumors has been observed, but once tumor and ulceration occur, the disease is considered aggressive, and the prognosis is poor.
The erythroderma stage can arise in isolation or progress from any other stage. The skin is diffusely red and scaly. The palms and soles may become very thick and crack, making it difficult to walk. There are other causes of erythroderma that should be excluded, including psoriasis, eczema, or a medication allergy.
With Sezary syndrome, there is generalized redness, scaling, intense itching, and lymph node involvement, as well as malignant cells circulating in the blood.