Diagnosis often requires a combination of clinical and pathological studies. Diagnosis is sometimes difficult because the early phases of the disease often resemble inflammatory dermatoses (such as eczema, psoriasis, lichenoid dermatoses including lichen planus, vitiligo, and chronic cutaneous lupus erythematosus), as well as other cutaneous lymphomas. Several biopsies are recommended, as the key microscopic features are often absent in early<unk> , and a complete diagnosis requires a combination of clinical and histological study. Furthermore, long periods of treatment can alter the biopsy findings, making it difficult to distinguish from other inflammatory dermatoses. Childhood<unk>  <unk>  makes up 0.5% to 7.0% of cases. Although data on childhood<unk>  is limited, a 2021 systematic review observed that there is a significant delay in the diagnosis of childhood<unk>  which may negatively affect a child’s prognosis. Notably, most pediatric persons with<unk>  present with early-stage disease. Histology Histopathology of Pautrier microabscesses in cutaneous T cell lymphoma. The criteria for the disease are established on the skin biopsy by the presence of the following: * Presence of cancer cells with twisted contours (cerebriform nuclei) * In the patch and plaque stages, the cancer cells are seen in the epidermis (the most superficial layer of skin). This is referred to as epidermotropism. *Pautrier's microabcesses, aggregates of four or more atypical lymphocytes arranged in the epidermis. Pautrier microabcesses are characteristic of<unk>  but are generally absent. *In the tumour stage, the cancer cells move into the dermis (the deeper layer of skin) *Large cell transformation, where clonally identical lymphocytes in the lesion exhibit hypertrophy. In transformed cells, presence of the CD30 receptor is associated with improved survival To stage the disease, various tests may be ordered, to assess nodes, blood and internal organs, but most patients present with disease apparently confined to the skin, as patches (flat spots) and plaques (slightly raised or 'wrinkled' spots). Peripheral smear will often show buttock cells. Staging Traditionally,<unk>  has been divided into three stages: premycotic, mycotic and tumorous. The premycotic stage clinically presents as an erythematous (red), itchy, scaly lesion. Microscopic appearance is non-diagnostic and represented by chronic nonspecific dermatosis associated with psoriasiform changes in epidermis. In the mycotic stage, infiltrative plaques appear and biopsy shows a polymorphous inflammatory infiltrate in the dermis that contains small numbers of frankly atypical lymphoid cells. These cells may line up individually along the epidermal basal layer. The latter finding if unaccompanied by spongiosis is highly suggestive of<unk> . In the tumorous stage a dense infiltrate of medium-sized lymphocytes with cerebriform nuclei expands the dermis. Accurate staging of<unk>  is essential to determine appropriate treatment and prognosis. Staging is based on the tumor, node, metastasis, blood (TNMB) classification proposed by the<unk>  Cooperative Group and revised by the International Society for Cutaneous Lymphomas/European Organization of Research and Treatment of Cancer. This staging system examines the extent of skin involvement (T), presence of lymph node (N), visceral disease (M), and presence of Sezary cells in the peripheral blood (B). Most patients with<unk>  have early-stage disease (Stage IA-IIA) at the time of their initial diagnosis. People with early stage disease that is primarily confined to the skin have a favorable prognosis. People with advanced stage (Stage IIB-IVB) are often refractory to treatment and have an unfavorable prognosis. Treatment options for people with advanced stage disease are designed to reduce tumor burden, delay disease progression, and preserve quality of life.
Mycosis fungoides
Sycosis vulgaris
Keratoderma climactericum
Tinea versicolor
Lymphomatoid papulosis
Actinic granuloma
Diabetic dermadrome
Granuloma faciale