Kaposi’s Sarcoma Resolves After Sirolimus Therapy in a Patient With Pemphigus Vulgaris

Abstract

Background: Iatrogenic Kaposi's sarcoma (KS) has been reported in patients who use immunosuppressive regimens for the treatment of autoimmune disorders, malignant neoplasms, and organ transplant rejection. However, iatrogenic KS in the setting of pemphigus vulgaris (PV) has been infrequently observed. The conventional treatment strategy for iatrogenic KS has focused on reducing immunosuppression, which carries a poor prognosis owing to a substantial risk for exacerbation of the primary disease. Observations: A 49-year-old man developed KS on his wrist after 2 years of long-term immunosuppressive therapy with prednisone, methotrexate, and dapsone for well-controlled PV. Three months after the substitution of methotrexate with sirolimus, the KS gradually resolved. With the patient on a maintenance regimen of sirolimus, in conjunction with low-dose prednisone and dapsone therapy, KS and PV have remained in remission, without further recurrence, during a 24-month follow-up period. Conclusion: The present case introduces a novel therapy for this patient population, highlighting the efficacy of sirolimus in treating iatrogenic KS without sacrificing the immunosuppression necessary to maintain control of PV.

Remission eines iatrogenen Kaposi-Sarkoms bei Myasthenia gravis nach Umstellung der Immunsuppression auf den mTOR-Inhibitor Everolimus

“…Kaposi sarcoma · Myasthenia gravis · Immunosuppression · mTOR inhibitor · Everolimus [19]. In einem anderen Fall konnte ein klassisches KS erfolgreich mit Sirolimus behandelt werden [10].…”

Stat3 regulates desmoglein 3 transcription in epithelial keratinocytes

“…Stat3 inhibition and rapamycin would similarly be predicted to have rapid therapeutic effect in PV, thus providing an alternative strategy to corticosteroids as first-line treatment. Consistent with this model, 2 prior cases of PV treated with systemic rapamycin reported rapid disease improvement (5,29). However, a case series of 3 patients with oral PV noted no effect of topical rapamycin (30).…”

Angiogenesis in cutaneous disease: Part I

“…145 Sirolimus has been used in treatment of anaplastic large cell lymphoma, KS, angiofibromas, and psoriasis. [146][147][148][149][150] Currently, there is a phase IV study to determine the effect of sirolimus on prevention of nonmelanoma skin cancers in kidney transplant recipients. There is also an active phase I study using topical sirolimus on basal cell nevus syndrome and a study evaluating use of sirolimus in combination with pulsed dye laser for treatment of port wine stain birthmarks.…”