CUTANEOUS T-CELL LYMPHOMAS (CTCL)
Tumors of Lymph nodes and Lymphatic system are called Lymphomas.
Extranodal lymphomas are the tumours that occur in organs or tissues outside the lymphatic system.
When lymphomas occur in the skin having no evidence of disease anywhere else at the time of diagnosis , they are called Primary Cutaneous Lymphomas .
There are many types of Primary Cutaneous Lymphomas, but two main categories are Cutaneous T- cell Lymphomas (CTCL) and Cutaneous B-Cell Lymphomas (CBCL). 65% of Cutaneous Lymphomas are T-Cell type.
The diagnosis is made by Skin Biopsy. CTCL is a serious and very uncommon skin condition in which there is an abnormal neoplastic proliferation of lymphocytes with a T- Subtype ( T is Thymus Derived).
CLASSIFICATION OF CTCL
- Mycosis Fungoides – The sub types are
- Folliculotropic MF
- Pagetoid Reticulosis
- Granulomatous Slack skin
- Primary Cutanoeus CD 30 + Lymphoproliferative Disorders
- Primary Cutaneous anaplastic large cell lymphoma
- Lymphomatoid papulosis
- Subcutaneous Panniculitic
- Primary Cutaneous CD4 + small/medium pleomorphic T-Cell Lymphoma, usually presented with a single lesion and progresses slowely.
In Primary Cutaneous Lymphomas, Mycosis Fungicides is the most common types of CTCL in 50 % of cases. The second most common is Primary Cutaneous CD30 + Lymph proliferative disorders.
The name mycosis is confusing and seems that it is a fungal infection. But it has nothing to with it. It is a condition in which the skin is infiltrated by patches or lumps composed of white cells called lymphocytes. Men are more common to have it than women and very rare in children. The cause of the disease is unknown , in some cases it may be associated with infections with retrovirus or pre-existing contact allergic dermatitis.
Mf may persists in one stage or over the year or sometimes decades , it slowly progress to another stage.
- PATCH STAGE
The lesions are flat in this stage, oval or ring shaped, pink dry patches on covered skin.
They may remain in the same size or slowly enlarge.
The skin may be atrophic , itching may or may not be there.
The differential diagnosis from Psoriasis , Discoid Eczema or ParaPsoriasis is difficult in this stage.
- PLAGUE STAGE
In this stage the patches become thickened and may resembles psoriasis. This stage is usually itchy.
- TUMOUR STAGE
In this stage of Mycosis Fungoides ,the large irregular lumps develop from plagues. Ulceration may occur in the lesions. This is a critical stage where it spread to the other organs . The tumour stage may transform to a large cell lymphoma.