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Skin cancer is the leading malignancy in immunosuppressed patients, including organ transplant recipients (OTRs), which is increasing in incidence as OTRs live longer. We performed a single-center case series of 4 patients with scalp pleomorphic dermal sarcoma and a history of multiple keratinocyte carcinomas. Outcomes included incidence of dermal sarcoma, dermal sarcoma-related mortality, and histopathologic findings. Out of more than 200 patients followed over a 3-year period in Massachusetts General Hospital High Risk Skin Cancer Clinics, all skin cancer-related deaths (2/2) were due to metastatic dermal sarcoma. Three of 4 patients diagnosed with scalp dermal sarcoma were OTRs and had been on at least one immunosuppressive medication for a median of 9 years. For patients who died from dermal sarcoma, the median time between diagnosis and death was 6 months. Our findings suggest pleomorphic dermal sarcoma contributes to skin cancer-related morbidity and mortality in OTRs.

Fibrosis is a pathological hallmark of systemic sclerosis, a deadly autoimmune disease affecting the connective tissues of multiple organs. However, the immune mechanisms underlying fibrosis and systemic sclerosis remain unclear. To determine the initiating immune pathway in fibrosis, we investigated the role of type 2 alarmin cytokines in the mouse model of skin fibrosis. Wild-type mice that received subcutaneous bleomycin injections developed skin fibrosis accompanied by elevated IL-33 expression in the dermis. Likewise, we found IL-33 upregulation in human skin fibrosis. Mice with germline deletion of IL-33 receptor (ST2 knockout) showed markedly exacerbated skin fibrosis in association with significantly increased T helper 2 cell to regulatory T-cell ratio in the skin. Mice that lacked ST2 specifically on regulatory T cells (Foxp3