Following consensus meetings of the two parent organizations, a new World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for primary cutaneous lymphomas has recently been published. This important development will now end the ongoing debate as to which of these was the preferred classification. The new classification will facilitate more uniformity in diagnosis, management and treatment of cutaneous lymphomas. In particular, it provides a useful distinction between indolent and more aggressive types of primary cutaneous lymphoma and provides practical advice on preferred management and treatment regimens. This will thereby prevent patients receiving high-grade treatment for low-grade biological disease. This review focuses on those diseases which have found new consensus agreement compared with the original WHO and EORTC classifications. In cutaneous T-cell lymphomas, these include folliculotropic mycosis fungoides, defining features of Sézary syndrome, primary cutaneous CD30+ lymphoproliferative disorders (primary cutaneous anaplastic large cell lymphoma, lymphomatoid papulosis and borderline lesions) and subcutaneous panniculitis-like T-cell lymphoma. Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma, primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma and cutaneous gamma/delta T-cell lymphoma are allocated provisional entry status and thereby afford better definitions for some cases of currently unspecified primary cutaneous peripheral T-cell lymphoma. In cutaneous B-cell lymphomas, diseases which have found new consensus agreement include primary cutaneous marginal zone B-cell lymphoma, primary cutaneous follicular centre lymphoma, primary cutaneous diffuse large B-cell lymphoma, leg type and primary cutaneous diffuse large B-cell lymphoma, other. CD4+/CD56+ haematodermic neoplasm (early plasmacytoid dendritic cell leukaemia/lymphoma) now appears as a precursor haematological neoplasm and replaces the previous terminology of blastic NK-cell lymphoma. Other haematopoietic and lymphoid tumours involving the skin, as part of systemic disease, will appear in the forthcoming WHO publication Tumours of the Skin. The new classification raises interesting new problems and questions about primary cutaneous lymphoma and some of these are discussed in this article. It is, however, a splendid signpost indicating the direction in which research in cutaneous lymphoma needs to go. In the interim, we have an international consensus classification which is clinically meaningful.
"Cytotoxic cutaneous lymphomas are a group of lymphoproliferative disorders characterized
by expression of cytotoxic proteins (TIA-1, granzyme A or B or perforin). 1,2 The diagnosis of these conditions can sometimes be clinically and
histopathologically challenging. "
[Show abstract][Hide abstract] ABSTRACT: Cytotoxic lymphomas comprise a spectrum of peripheral T-cell lymphomas that can have
a initial or late cutaneous presentation. We describe a 46-year-old man from Cape
Verde, with a dermatosis involving his face and trunk, consisting of monomorphic
papules with a smooth surface and both motor and sensory polyneuropathy.The
hypothesis of leprosy was supported by the clinical and initial hystopathological
findings and the patient was referred to our hospital with suspected Hansen's
disease. In the new skin and lymph node biopsies a lymphocyte population was
identified whose immunohystochemistry study allowed the diagnosis of T-cell lymphoma
with expression of cytotoxic markers. The patient was started on chemotherapy with
initial remission of the skin lesions but, subsequently, progression of systemic
"It was not until the middle of the first decade of the current century that the term " primary cutaneous marginal-zone lymphoma " encompassed primary cutaneous immunocytoma and primary cutaneous plasmacytoma    "
[Show abstract][Hide abstract] ABSTRACT: The current report examines the evolution of the concepts of immunocytoma and pseudolymphoma in a historical perspective, paying special attention to their evolvement into the groups of marginal-zone lymphoma and cutaneous MALT-lymphoma. It also examines the current conception of the existence of at least two types of cutaneous MALT-lymphomas and their relation to the duality immunocytoma/pseudolymphoma from the old literature.
Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie 03/2013; 54(1):7-12. · 0.66 Impact Factor
"Differentiation between these two conditions is, however, important because patients with lymphomatoid papulosis, unlike those with pityriasis lichenoides, may develop systemic lymphoma . The presence of large CD30+ atypical lymphoid cells is the hallmark of lymphomatoid papulosis . Other differences include the presence of large, atypical, non-lymphoid cells that may resemble Reed-Sternberg cells, many neutrophils, few lymphocytes, few or no necrotic keratinocytes, and little or no vacuolar degeneration of the basal layer, observed in lymphomatoid papulosis as opposed to PLEVA . "
[Show abstract][Hide abstract] ABSTRACT: We report a case of a 63-year-old man hospitalized for a polymorphous generalized eruption consisting of maculopapules with peripheral scaling, vesicopustules, and ulceronecrotic and crusted lesions measuring 5-20 mm, localized on his trunk and extremities, particularly exuberant in the flexural area. Histopathology showed necrotic keratinocytes with exocytosis of red blood cells and lymphocytes and a dermal perivascular and periadnexal inflammatory infiltrate, composed of CD8+/CD4-/CD30- T cells, indicating the clinical diagnosis of pityriasis lichenoides et varioliformis acuta. He was treated with erythromycin and methylprednisolone reduced gradually over 5 months, with a slow but complete response; the patient was without lesions after 2 years of follow-up. The authors want to remind of this rare entity which may present difficulties in diagnosis and therapy.
Case Reports in Dermatology 03/2012; 4(1):61-5. DOI:10.1159/000337745
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