[Cutaneous Rosai-Dorfman syndrome. Successful therapy with intrralesional corticosteroids]. / Kutanes Rosai-Dorfman-Syndrom. Erfolgreiche Therapie mit intraläsional applizierten Steroiden.

BACKGROUND: Cutaneous Rosai-Dorfman disease is a rare disorder belonging to the spectrum of non-Langerhans cell histiocytoses. It is characterized by dermal and subcutaneous infiltrates of histiocytes as well as accompanying lymphocytes, plasma cells and granulocytes. Because it is so rare, standard therapies have not been established. CASE REPORT: A 27-year-old man showed an excellent response to intralesional corticosteroids after unsuccessful prior treatment with methotrexate, systemic steroids and surgery as well as laser therapy.

Long-term outcome of intravenous therapy with rituximab in patients with primary cutaneous B-cell lymphomas.

BACKGROUND: The monoclonal antibody rituximab directed against the B-cell antigen CD20 was approved for the treatment of B-cell lymphomas and maintenance therapy in follicular lymphomas more than a decade ago. However, median follow-up in case series of intravenous rituximab therapy in primary cutaneous B-cell lymphomas (CBCL) lasts only up to 3 years. We retrospectively analysed a cohort of CBCL patients treated with rituximab to gain more long term information. PATIENTS AND METHODS: Eighteen patients, treated intravenously with rituximab for a primary cutaneous B-cell lymphoma [follicle centre lymphoma (PCFCL), n = 11; diffuse large B-cell lymphoma, leg type (PCLBCL, leg type), n = 5; marginal zone B-cell lymphoma (PCMZL), n = 2] were included. The response rate (RR), time to relapse (TTR), and course of the disease after treatment were analysed. RESULTS: The overall RR was 89% (16 of 18 patients). Within the median follow-up time of 52 months, 81% (13 of 16) of patients experienced a relapse; the median TTR was 25 months. The duration of remission was significantly shorter in patients presenting with generalized skin lesions at start of therapy. Both nonresponding patients suffered from PCLBCL, leg type, with extracutaneous manifestations. In responders severe adverse events, the occurrence of extracutaneous dissemination or nodal lymphomas were not observed during follow-up. CONCLUSIONS: Therapy with rituximab is effective and safe for the treatment of PCFCL, but relapses, in particular in patients with generalized skin involvement, are commonly observed. However, all patients with relapses responded well to treatment and therefore maintenance therapy does not seem to be indicated. Patients with PCLBCL, leg type, should receive chemotherapy in addition to rituximab.

[CD30-positive anaplastic large cell T-cell lymphoma developing during immunosuppressive therapy of pityriasis rubra pilaris with ustekinumab]. / CD30-positives anaplastisch großzelliges T-Zell-Lymphom unter immunsuppressiver Therapie einer Pityriasis rubra pilaris mit Ustekinumab.

The development of malignancies during therapy with biologics has been discussed controversially. A patient with extensive pityriasis rubra pilaris failed to respond to standard therapeutic approaches. While receiving immunomodulatory therapy, lastly with ustekinumab, the patient developed a CD30(+) anaplastic large cell lymphoma.

[Cutaneous malignant lymphomas. Update on diagnosis and therapy of cutaneous T-cell lymphomas]. / Maligne Lymphome der Haut. Update zu Diagnostik und Therapie kutaner T-Zell-Lymphome.

Cutaneous T-cell lymphomas represent extranodal non-Hodgkin lymphomas of mature T-cells, which accumulate in the skin. They have been recognized as a heterogeneous group with distinct variability in clinical presentation and histopathology, with divergent biological behaviour and prognosis. Therefore the exact diagnosis is an important prerequisite for an adequate and stage-adapted treatment.

Evaluation of different methods in the follow-up of patients with indolent types of primary cutaneous lymphomas.

BACKGROUND: Primary cutaneous lymphomas (CLs) are a heterogeneous group of diseases arising from B or T lymphocytes. CLs are grouped according to their clinical behaviour into indolent, intermediate and aggressive types. Indolent CLs respond well to therapy but frequently relapse, resulting in prolonged periods of follow-up. OBJECTIVES: To evaluate the outcome of follow-up examinations in indolent CL. METHODS: We retrospectively analysed a cohort from a CL outpatient clinic at a tertiary referral centre. Seventy-five patients with indolent cutaneous T-cell lymphomas (CTCLs) and 34 patients with indolent cutaneous B-cell lymphomas (CBCLs) were included. The value of clinical examination, blood tests and imaging procedures for detection of recurrence or progression was assessed. RESULTS: In patients with CTCL all but one disease recurrences were detected by clinical examination. Lymph node or organ involvement was detected by imaging procedures in seven patients, of whom all but one had recurrent or persistent CL lesions. In CBCL all recurrences were detected by clinical examination. CONCLUSIONS: Patients with indolent CL confined to the skin should be followed primarily by clinical examination. However, in patients who are refractory to treatment regular screening of lymph nodes by ultrasound may enable earlier detection of disease recurrence or progression.

Diagnostic tools in mycosis fungoides.

Cutaneous T-cell lymphomas (CTCL) represent clonal proliferations of neoplastic skin homing T-cells. Within the group of primary CTCL, mycosis fungoides (MF) is the most common entity, affecting the skin as a primary site. MF initially presents in the skin with a slow indolent course of a characteristic stepwise progression from patches to plaques and tumors accompanied by distinctive histological changes. Routine diagnosis is based on these clinical and histological features. However, due to similarities with benign lymphoproliferative or reactive skin diseases, especially at the initial presentation of the disease, diagnosis can be difficult. Although the etiology of mycosis fungoides is still unknown, important insights have been gained in the immunological and genetic perturbations, which are associated with the disease. In the last years the emergence of molecular genetic techniques allowing to analyze the clonality status in lymphocytic infiltrates, has provided new tools with the potential to increase the accuracy of diagnosis, staging and therefore stage-adapted treatment. Nevertheless, it is important to notice that some limitations restrict the predictive value of the results obtained by these analyses. Diagnostic tool of MF, including clinical, histo- and immunohistological findings as well as molecular genetic analysis will be covered in this review.