[Clinical experience with efalizumab in the Hospital of Cruces]. / Experiencia clínica con efalizumab en el Hospital de Cruces.

Efalizumab (Raptiva) is one of the biological agents approved recently for the treatment of patients with moderate-severe psoriasis who have not responded to conventional treatments. It is a humanized IgG1 monoclonal antibody which, due to its anti-D11 effect, is capable of blocking the endothelial migration and T cell activation on the skin, fundamental processes in the etiopathogeny of psoriasis. We present the experience we have had in our hospital over the last two years with 23 patients who have initiated treatment with efalizumab.

[Autologous hematopoietic stem cell transplantation followed by oral bexarotene in a patient with advanced mycosis fungoides]. / Trasplante autólogo de progenitores hematopoyéticos seguido de bexaroteno oral en paciente con micosis fungoide avanzada.

We describe the case of a 17-year-old patient with rapidly progressing and aggressive mycosis fungoides, with multiple cutaneous tumors and large cell transformation. She was initially treated with 3 cycles of high-dose chemotherapy with mega-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) without response, leading to the decision to undertake autologous hematopoietic stem cell transplantation. Partial remission of the disease was achieved with this treatment and subsequent introduction of oral bexarotene led to complete remission, which has been maintained for more than 3 years with good tolerance of oral therapy. We discuss the advantages and disadvantages of autologous hematopoietic stem cell transplantation and the use of oral bexarotene.

Successful treatment of musk ketone-induced chronic actinic dermatitis with cyclosporine and PUVA.

We describe a patient with chronic actinic dermatitis whose photopatch tests revealed reactions to musk ketone and musk ambrette, both of which were found in his aftershave lotion. Minimal erythema doses of UVA and UVB were decreased. After initial unsuccessful treatment with PUVA therapy the patient was successfully treated with a combination of cyclosporine and PUVA.

[Familial eosinophilic fascitis induced by toxic oil]. / Fascitis eosinofílica familiar inducida por aceite tóxico.

Three of four family members in husband, wife and one of two daughters developed in a short time interval (eleven months) eosinophilic fasciitis. The clinical, analytical and histopathological changes were characteristic of this disease. Familial cases of eosinophilic fasciitis have not been previously published. The process of these patients was probably caused by the ingestion of denatured oil (toxic oil syndrome), although the clinical picture begun two and a half years after the epidemic phase of the toxic oil syndrome declined.

Pre-Sézary syndrome.

Eighteen patients with erythroderma, recurrent cycles of circulating Sézary cells of less than 1,000 cells/mm3, and a chronic course were followed for a mean time of nearly 5 years and were diagnosed as having pre-Sézary syndrome. Only one patient died, and none developed lymphoproliferative disease. All ten patients who underwent patch testing showed positive results. The elevation of IgE was striking when this group was compared with a group with Sézary syndrome. Most patients achieved partial or complete remission on low-dose chlorambucil and prednisone therapy. Some patients had lymphocytic or lymphomatoid bands on skin biopsy specimens and were like previously reported patients with pre-Sézary syndrome whose condition progressed to Sézary syndrome. A nontoxic chemotherapy or an anti-T cell treatment program can control this chronic erythroderma state.

The treatment of Sézary syndrome.

The results of treatment programs were studied in forty patients with Sézary syndrome. Thirty-three patients died. The principal treatment was a low-dose chlorambucil and prednisone regimen. Patients so treated lived longer from diagnosis of erythroderma (median survival, 6.2 yr) than did patients on other regimens (median survival, 3.05 yr). The addition of x-ray or chemotherapeutic programs did not increase significant long-term benefit. Seven patients receiving chlorambucil and prednisone had remission (1 yr or more); most patients had partial remission, but two patients did not have a response to this program. The complications of sepsis, progressive disease, and lymphoma occurred both in patients who were and in those who were not treated with chlorambucil and prednisone. We believe that a regimen of low-dose chlorambucil and prednisone is satisfactory treatment that can be the basis for comparison of new therapeutic approaches to Sézary syndrome.