Letter to the Editor
Mantle cell lymphoma with skin involvement

Mantle cell lymphoma (MCL) is a rare disease of the lymphomatoid system arising from mature B lymphocytes and comprises 3–10% of all non-Hodgkin's lymphoma subtypes and typically involves lymph nodes. According to the guidelines of the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC), the diagnosis of MCL should be established on the basis of morphological examination and immunophenotyping with detection of cyclin D1 protein overexpression and/or chromosomal translocation t(11,14)(q13;q32) of the CCND1 gene [1]. Histologically MCL is composed of diffuse or nodular proliferations of B lymphocytes in the mantle zone of lymphoid follicles in lymph nodes with characteristic immunophenotypical pictures positive for B-cell markers, like CD79a, CD19, CD20, CD22 and CD5 as well as usually negative for CD10, CD23 and bcl-6. The blastoid variant of MCL develops in 10–30% of patients with classic MCL [2]. This type of lymphoma joints two inauspicious clinical features: incurability and quick progression. At the time of diagnosis, the disease is usually in the advanced stage with a frequent generalized lymphadenopathy, splenomegaly and the bone marrow, blood or gastrointestinal involvement (stage III, IV in Ann Arbor scale) [3]. The disease generally involves lymph nodes but primary extranodal localization may also occur especially in the bone marrow, spleen, gastrointestinal tract and Waldeyer's ring. In the classification of WHO-EORTC, mantle cell lymphoma is listed as an extracutaneous lymphoma secondarily involving the skin. However, skin involvement, if present, is usually common in the widespread disease [4]. The primary skin involvement in MCL is extremely rare and controversial [5].
We report a case of a patient with MCL secondary involving the skin and review of the literature of cutaneous involvementinpreviously reported cases of MCL.
A 63-year-old woman referred to the Department of Dermatology presented with severe thick erythematous infiltrations located on all her face and the ears as well as hyperpigmentations on the upper part of the trunk. Skin lesions on the face started as erythema resembling photosensitivity and progressively enlarged (Figures 1 A–B). On admission the patient has had the dryness of the oral mucosa, difficulties in speaking, increased dyspnea for several weeks. The generalized lymphadenopathy was observed. Laboratory abnormalities included an elevated level of leucocyte...


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