Cutaneous lymphomas: A review

Abstract

SUMMARYSkin may be affected by non-Hodgkin lymphomas, and it is the second most frequently involved extranodal organ, after the gastrointestinal tract. Cutaneous lymphomas may originate from T, B, or NK lymphocytes. Diagnosis is difficult, and knowledge of these diseases is important to ensure their detection and adequate treatment and follow-up. Clinical picture is heterogeneous, and histology is essential to confirm the diagnosis. The classification is given by the correlation between clinical findings, histology and immunophenotyping. Therapeutic options include skin-directed therapies, immunomodulatory or low-dose immunosuppressive drugs. Rare cases of aggressive disease require systemic multidrug chemotherapy. The purpose of this review is to describe the ontogeny of T, B and NK lymphocytes, and to detail the most accepted classification of cutaneous lymphomas, proposed by the World Health Organisation and European Organisation for Research and Treatment of Cancer. Treatment of cutaneous lymphomas will be briefly discussed.

Cutaneous T-Cell Lymphoma: Establishing a Diagnosis in a Patient with a Dramatic Tumor

“…Primary cutaneous lymphomas have an incidence of 1 per 100,000 in the United States with primary cutaneous T-cell lymphomas outnumbering B-Cell lymphomas at a rate of three to one [1]. Males are affected more commonly with average age of onset after 50 years.…”

“…Males are affected more commonly with average age of onset after 50 years. Mycosis fungoides, also known as Alibert-Bazin syndrome, is the most prevalent of the CTCLs, accounting for up to 50% of cases [1]. Although the etiology is unknown, multiple possible triggers, including genetic, environmental, and infectious, have been postulated.…”

“…Prognosis is directly related to subtype and tumor stage [6]. With classic MF, survival is inversely related to the stage of the disease [1,3]. Five-year survival for classical MF is up to 97.3% when <10% of the BSA is affected by patches and plaques, but declines to 27.6% if tumorous lesions exist [1].…”

“…With classic MF, survival is inversely related to the stage of the disease [1,3]. Five-year survival for classical MF is up to 97.3% when <10% of the BSA is affected by patches and plaques, but declines to 27.6% if tumorous lesions exist [1]. Granulomatous slack skin disease has an excellent prognosis, in contrast to folliculotropic MF, which portends to the worst prognosis with 5-year survival rates between 60% and 70% [3].…”

“…Mycosis fungoides (MF), the most common subset of cutaneous T-cell lymphoma (CTCL) [1], is one of the great masqueraders in medicine. Despite this, however, the clinical picture can be striking, and persistence is often needed in confirming the diagnosis [1,2]. We present a case of dramatic tumor stage mycosis fungoides.…”