Complete remission of scleredema adultorum of Buschke associated with multiple myeloma after bortezomib-based treatment and autologous peripheral blood stem cell transplantation

摘要

A 53-year-old male presented with progressive asymptomatic cutaneous hardening on the nape of his neck for 6 months. The lesion had an insidious onset and caused the patient to experience impaired movement of the neck. Physical examination disclosed a symmetric woody-hard, nonpitting indurated erythematous plaque on the nape of his neck [Figure 1]a. A skin biopsy from the nape showed thickened reticular dermis with dermal fenestration [Figure 2]a and [Figure 2]b. Abundant mucin deposition in the deep dermis was demonstrated by Alcian blue stain [Figure 2]c. Scleredema adultorum of Buschke was therefore diagnosed. Laboratory data revealed anemia (9.7 g/dl) and hypercalcemia (10.2 mg/d). His renal function (creatinine: 1.09 mg/dL; range: 0.64–1.27), glycated hemoglobin (6.0%; range: 5.7–6.4), complement 3 (90 mg/dL; range: 90–180), and complement 4 (39 mg/dL; range: 10–40) were within the normal limits. Both anti-nuclear antibody and anti-streptolysin were negative. Serum protein electrophoresis showed a monoclonal band in the γ region and immunofixation confirmed a monoclonal immunoglobulin A and κ light chain. Radiographic skeletal survey showed diffuse osteopenia. In particular, multiple radiolucent lesions were seen at the skull and eighth thoracic spine. A bone marrow biopsy [Figure 2]d and [Figure 2]e showed increased plasma cells (80%) and staining of κ light chain proteins. A diagnosis of scleredema adultorum of Buschke in association with multiple myeloma of IgA-κ was made. The patient was started on VTD regimen (Bortezomib [Velcade] 1–1.3 mg/m2 subcutaneous on days 1, 4, 7, and 11; Thalidomide 100 mg perorally daily; Dexamethasone 40 mg perorally daily, four cycles). Subsequently, he received autologous peripheral blood stem cell transplantation after a preconditioning of high-dose melphalan. The treatment resulted in the rapid decline of monoclonal IgA with complete disappearance of paraprotein. The skin induration gradually improved, and he regained full neck mobility after 9 months of therapy [Figure 1]b. The patient remained disease-free at 24-month follow-up.