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Waxy Indurated Plaques on the Eyelids

Cutis. 2015 February;95(2):E24-E27
February 23, 2015|Cutis
Maria A. Aleshin, MD;Trent D. Custis, MD;Nima M. Gharavi, MD, PhD
Author and Disclosure Information

Maria A. Aleshin, MD; Trent D. Custis, MD; Nima M. Gharavi, MD, PhD; Carolyn Goh, MD; Lorraine C. Young, MD

Dr. Aleshin is from the Department of Medicine, and Drs. Gharavi, Goh, and Young are from the Division of Dermatology, all at the University of California at Los Angeles David Geffen School of Medicine. Dr. Custis is in private practice, Dallas, Texas.

The authors report no conflict of interest.

Correspondence: Lorraine C. Young, MD, Department of Medicine, University of California at Los Angeles (UCLA) David Geffen School of Medicine, 200 UCLA Medical Plaza, Ste 450, Los Angeles, CA 90095 (lcyoung@mednet.ucla.edu).

Figure 2. Ventral aspect of the thumb with extensive nonpalpable purpura.

Figure 3. Shoulder with extensive nonpalpable purpura.

Treatment

Treatment regimens for AL amyloidosis have historically been adapted from those used to treat multiple myeloma, with the primary focus being the reduction of the amyloidogenic clone. The first effective therapy for AL amyloidosis was melphalan and prednisone. Although this regimen extended survival from half a year (6–8 months) to a year (12–18 months), it also was plagued by severe side effects and delayed response times. Increased response rates and fewer side effects have been achieved by replacing prednisone with dexamethasone in the aforementioned regimen.15 High-dose melphalan conditioning followed by autologous stem cell transplant has shown tremendous promise, with complete remission rates approaching 40% and median survival exceeding 4 years in some studies.16 However, the treatment-related mortality of stem cell transplant regimens is 10% to 20%, thus limiting this approach to younger and healthier patients. Exciting early work is emerging to support the efficacy of novel agents in the treatment of AL amyloidosis, including thalidomide, lenalidomide, and bortezomib. Because these agents do not have many of the traditional side effects associated with chemotherapy, they are increasingly being used in patients who are not candidates for stem cell transplant or melphalan-dexamethasone. They also can be used as salvage therapy in patients with relapsed disease.

Conclusion

Primary AL amyloidosis is a rare disorder that carries a poor prognosis. One of the major challenges for clinicians is diagnosis, as nearly any organ system can be affected, resulting in variable presentations. Although cutaneous manifestations are highly characteristic of AL amyloidosis and may help aid in its diagnosis, only a limited number of patients have involvement of the skin. As such, further research is needed to facilitate earlier diagnosis of primary AL amyloidosis as well as to develop novel therapies for this devastating disease.

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