Case Report/Case Series |

Long-term Plasmapheresis in Conjunction With Thalidomide and Dexamethasone for the Treatment of Cutaneous Ulcers and Neovascular Glaucoma in Recalcitrant Type I Cryoglobulinemia

Chao H. Yang, BS1,2; Abrar A. Qureshi, MD, MPH1; Winthrop H. Churchill, MD3; Arturo P. Saavedra, MD, PhD, MBA1
[+] Author Affiliations
1Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts
2currently a student at Harvard Medical School, Boston, Massachusetts
3Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts
JAMA Dermatol. 2014;150(4):426-428. doi:10.1001/jamadermatol.2013.8700.
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Importance  Cryoglobulins are cold-precipitating immunoglobulins that occur secondary to lymphoproliferative disorders, chronic viral infections, and autoimmune disorders. The treatment of cryoglobulinemia should target the underlying disorder; however, such an approach may be difficult, and therapeutic options remain limited for type I cryoglobulinemia.

Observations  We report a case of recalcitrant type I cryoglobulinemia treated successfully with long-term plasmapheresis in conjunction with thalidomide and dexamethasone. A woman in her 50s with cryoglobulinemia and bilateral lower extremity ulcers of 1 year’s duration developed acute angle-closure glaucoma following the appearance of new macules on her upper extremities. An initial short course of 5 plasmapheresis treatments improved the patient’s cutaneous lesions as well as the glaucoma. Three weekly doses of rituximab were not associated with any evidence of clinical improvement, so thalidomide and dexamethasone were administered as replacement therapy. Because of the increasing pain and persistence of the woman’s ulcers, intensive plasmapheresis was resumed and continued 3 to 4 times per week for approximately 4 months, after which a slow tapering regimen was initiated. This therapy was associated with progressive, rapid healing of the ulcers, stabilization of the skin lesions, and control of the patient’s intraocular pressure.

Conclusions and Relevance  The long-term use of plasmapheresis may be a well-tolerated treatment option for therapeutically challenging cases of cryoglobulinemia.

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Figure 1.
Lower Extremity Ulcers Before Initiation of Long-term Plasmapheresis Treatment

Nonpalpable purpura, eschar formation, and large ulcers were present in the pretibial and ankle regions.

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Figure 2.
Hematoxylin-Eosin Staining of the Left Lower Extremity Skin Biopsy

Notable findings included capillary vascular proliferation and intravascular periodic acid–Schiff–positive material (original magnification ×40).

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