Cutaneous primary B-cell lymphomas: from diagnosis to treatment

Primary cutaneous B-cell lymphomas are a heterogeneous group of mature B-cells neoplasms with tropism for the skin, whose biology and clinical course differ significantly from the equivalent nodal lymphomas. The most indolent forms comprise the primary cutaneous marginal zone and follicle center B-cell lymphomas that despite the excellent prognosis have cutaneous recurrences very commonly. The most aggressive forms include the primary cutaneous large B-cell lymphomas, consisting in two major groups: the leg type, with poor prognosis, and others, the latter representing a heterogeneous group of lymphomas from which specific entities are supposed to be individualized over time, such as intravascular large B-cell lymphomas. Treatment may include surgical excision, radiotherapy, antibiotics, corticosteroids, interferon, monoclonal antibodies and chemotherapy, depending on the type of lymphoma and on the type and location of the skin lesions. In subtypes with good prognosis is contraindicated overtreatment and in those associated with a worse prognosis the recommended therapy relies on CHOP-like regimens associated with rituximab, assisted or not with local radiotherapy. We review the primary cutaneous B-cell lymphomas, remembering the diagnostic criteria, differential diagnosis, classification, and prognostic factors and presenting the available therapies.

Childhood Non-Langerhans Cell Histiocytosis – An Atypical Case

Introduction: Histiocytoses correspond to a heterogeneous group of disorders characterized by the proliferation andinfiltration of histiocytes in tissues. For years, many of the histiocytoses where known by numerous names, reflecting the lack of understanding regarding their origin. Case report: A previously healthy, 4-month-old infant presented a cutaneous lesion of progressive growth on his nose since 1 month ago. The mother has a diagnosis of Sjögren syndrome. On examination, there was a firm well-defined erythematous plaque with 30 mm of diameter and raised edges, on the bridge of the nose. At the left cheek, left thoracic region and right arm there were also small erythematous papules with 5 days of evolution. The analytical study showed elevated antinuclear antibodies and anti-SS-A. Histopathological examination confirmed the diagnosis of non-Langerhans cells histiocytosis. Progressively, more annular erythematous papules and plaques appeared scattered over the face, trunk and limbs with spontaneous resolution of the first lesion on the nose. About 2 months after the initial evaluation, all lesions had regressed spontaneously, with normalization of analytical study at one year-old. Conclusion: Many authors believe that the different subtypes of histiocytosis represent only different stages of the same disease. Immunophenotyping of histiocytosis does not always identify the subtype involved, making the occurrence of atypical cases frequent.