ABSTRACT
There is an array of primary skin disorders that have been considered autoimmune diseases in their pathogenesis. Most of them are proven to have pathogenic autoantibodies that react against specific antigens on the cell surface of the keratinocytes or basement membrane components. Patients with autoimmune blistering diseases are commonly treated with systemic corticosteroids and immunosuppressant agents. Although most patients can be controlled with standard medications, a minority of patients have a severe clinical course and are resistant to conventional treatment. Most of these patients succumb from the complications of treatment, especially long-term corticosteroids. Recently, “immunoablation” with high dose chemotherapy followed by autologous hematopoietic transplantation has demonstrated that it can induce durable remissions for severe autoimmune diseases. 1 For autoimmune bullous skin diseases, case reports of high-dose cyclophosphamide without stem cell support also suggest promise as a treatment option. 2 In patients with extensive denuded skin lesions who have open skin lesions, a fear of serious infection during the neutropenic period after sub-myeloablative chemotherapy is a concern. Autologous hematopoietic stem cells can minimize the duration of the cyclophosphamide-induced neutropenic period.
