Ctep

Cutaneous T-cell lymphoma
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Skin lesions, plaques, tumors
Complications	Infection, systemic spread
Onset
Duration
Types	N/A
Causes
Risks
Diagnosis	Skin biopsy, blood tests
Differential diagnosis	N/A
Prevention	N/A
Treatment	Phototherapy, chemotherapy, radiation therapy
Medication	N/A
Prognosis	Variable
Frequency	Rare
Deaths	N/A

Cutaneous T-cell lymphoma (CTCL) is a type of non-Hodgkin lymphoma that primarily affects the skin. It is characterized by the presence of malignant T-cells in the skin, leading to various dermatological manifestations. CTCL is a rare condition and represents a group of lymphoproliferative disorders.

Classification

CTCL encompasses several subtypes, the most common of which are:

• Mycosis fungoides
• Sézary syndrome

Mycosis Fungoides

Mycosis fungoides is the most prevalent form of CTCL. It typically presents with patches, plaques, and tumors on the skin. The disease progresses slowly and can evolve over years or decades.

Sézary Syndrome

Sézary syndrome is a leukemic form of CTCL characterized by the triad of erythroderma, lymphadenopathy, and the presence of malignant T-cells in the blood. It is more aggressive than mycosis fungoides.

Pathophysiology

CTCL arises from the clonal proliferation of T-lymphocytes, specifically CD4+ T-cells. These malignant cells infiltrate the skin, leading to the characteristic lesions. The exact cause of CTCL is unknown, but genetic and environmental factors may play a role.

Clinical Presentation

The clinical presentation of CTCL varies depending on the subtype and stage of the disease. Common symptoms include:

• Patches: Flat, scaly areas of skin that may be itchy.
• Plaques: Raised, thickened areas of skin.
• Tumors: Nodular lesions that may ulcerate.
• Erythroderma: Widespread redness and scaling of the skin.

Diagnosis

The diagnosis of CTCL is based on clinical evaluation, skin biopsy, and laboratory tests. Key diagnostic procedures include:

• Skin biopsy: Histopathological examination reveals atypical T-cells in the epidermis and dermis.
• Immunohistochemistry: Used to identify specific markers on T-cells.
• Flow cytometry: Analyzes the phenotype of circulating T-cells in the blood.

Treatment

Treatment of CTCL depends on the stage and severity of the disease. Options include:

• Topical corticosteroids: Used for early-stage disease to reduce inflammation.
• Phototherapy: Narrowband UVB or PUVA therapy can be effective for skin lesions.
• Systemic therapy: Includes oral retinoids, interferons, and targeted therapies.
• Chemotherapy: Used for advanced or refractory cases.
• Radiation therapy: Localized radiation can be used for specific lesions.

Prognosis

The prognosis of CTCL varies widely. Early-stage mycosis fungoides has a good prognosis with appropriate treatment, while advanced stages and Sézary syndrome have a poorer outlook. Prognostic factors include the extent of skin involvement, presence of blood involvement, and response to treatment.

Epidemiology

CTCL is a rare disease, with an incidence of approximately 6 cases per million people per year. It is more common in males and typically presents in individuals over the age of 50.

See Also

• Lymphoma
• Dermatology
• Oncology

Classification
External resources