9th July 2023

Other Neoplasms

Mycosis Fungoides

The most common type of primary cutaneous T-cell lymphoma (CTCL) in which clonal T lymphocytes accumulate in the skin. Presentation is variable with different sized heterogeneous erythematous patches and plaques. Diagnosis is challenging and requires blood tests, biopsies, and specialist input. It is not curable, and relapses are common after treatment.

Sezary Syndrome

A rare leukaemic variant of CTCL in which atypical T cells (Sezary cells) accumulate in the skin, blood, and lymph nodes. It presents as pruritic erythroderma (≥90% body surface affected) and lymphadenopathy.

Systemic Mastocytosis

Clonal neoplastic mast cells proliferate and accumulate in bodily tissues such as the skin, bone marrow, lymph nodes and other organs. Mast cell activation releases histamine and triggers an inflammatory response. Cutaneous involvement can present with itch, swelling and erythema. Systemic involvement can present in various ways, such as hypotension, flushing and abdominal pain. Serum tryptase levels may be raised and a tissue biopsy confirms the diagnosis.