Aims: To assess the efficacy and safety of rituximab in refractory pemphigus and the possible benefit of an additional prophylactic infusion at 6 months. Methods: Seventeen patients with pemphigus vulgaris, 1 with pemphigus foliaceus and 1 with pemphigus vegetans were treated with 4 weekly infusions of rituximab (375 mg/m2). Nine patients received an additional prophylactic infusion after 6 months while the rest received no maintenance therapy. In case of recurrence, an additional single infusion was administered. Results: Control of the disease was obtained after 3-8 weeks. End of the consolidation phase for all patients was observed after 16 weeks. Patients remained in full remission for 7-42 months. All immunosuppressive agents, including prednisone, were discontinued after 2-12 months. The disease relapsed in 5 out of 9 patients who received the additional prophylactic infusion, and in 3 out of 10 patients among those skipping the prophylactic additional infusion. Conclusion: One course of rituximab and treatment of relapses is highly effective and well tolerated in the treatment of refractory pemphigus. In this pilot study of 19 patients, the prophylactic infusion does not appear to have provided any additional benefit to the patients receiving it.
A 54‐year‐oid woman presented with two nonsymptomatic subcutaneous nodules. Approximately 3 months previously, after sustaining a fall, her X‐rays revealed a pathologic fracture and a highly destructive appearing lesion. She underwent amputation of the right leg up to the middle of the femoral bone. Her history was negative for any pre‐existing bone condition and her famiiy history was negative for malignant diseases. The pathology report macroscopically showed a neoplastic tumor with a maximal diameter of 12 cm which literally occupied almost all of the knee arthrosis and extended into the femoral condylus. Microscopic examination of the specimen showed a high grade malignant sarcoma comprising mainly spindle cells with a large percentage of necrosis. The exact histologic typing was uncertain, but was probably fibroblastic‐type osteosarcoma (Fig. 1).
On admission, her physical examination was significant for the presence of two subcutaneous nodules. One nodule was located on the abdominal wall in the lower left area (approximately 1×1 cm) and the other on the apex area of the scalp (approximately 1.5x1.5 cm). These lesions were not ulcerated and there was no change in skin pigmentation. The patient mentioned that they appeared a couple of months ago. She also complained of recent dim vision in the left eye.
Laboratory findings included a positive fine needle aspiration (FNA) of both nodules showing metastatic infiltration of the skin and subcutaneous tissue by osteosarcoma (Fig. 2), as well as a positive computed tomography (CT) scan of the orbita showing a metastatic tumor retro‐ocularly to the left which pressed against the optic nerve and was in contact with the lateral rectus. It is noteworthy to point out that, in this patient, both chest roentgenogram and CT of the chest were negative. Bone scan, liver ultrasound, and CT of the upper and lower abdomen were also negative. All other laboratory findings were within normal limits.
The decision was made that the patient was to undergo chemotherapy consisting of two cycies of Adriamycin (ADR) and Piatamine (CDDP), 21 days apart, followed by one cycle of BCD (Bleomycin, Cyclophosphamide, and Dactinomycin) after 21 days. This regimen was to be repeated once for a total of six cycles, each 21 days apart.
One day prior to the commencement of chemotherapy, a third subcutaneous nodule presented on the right side of the back in the scapular area. This also had a positive FNA showing metastatic osteosarcoma.
On admission of the patient for the second cycle of chemotherapy (ADR and CDDP), a fourth nodule was discovered on the upper third of the left thigh, and on admission for the third cycle (BCD), a fifth nodule was found on the left arm (brachium area). The patient's‐chest roentgenogram continued to be negative.
A little over ½ months after initiating chemotherapy, on entering the fourth cycle (ADR and CDDP), it was observed that two of the five nodules (on the left arm and in the left lower abdominal area) had disappeared. There was also a decrease in the size of the...
Although the association of seborrhoeic keratosis and skin malignancy appears to be relatively uncommon, the possibility of such an association cannot be ruled out.
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