mycophenolic-acid and Stevens-Johnson-Syndrome

Severe bullous eruptions in systemic lupus erythematosus (SLE) patients include bullous SLE, Rowell syndrome, toxic epidermal necrolysis (TEN), and TEN-like eruption of acute cutaneous lupus (TEN-like ACLE). TEN-like ACLE, a rare manifestation of SLE that closely mimics TEN, can be distinguished by characteristic clinical and laboratory findings. A 27-year-old man with SLE who developed TEN-like ACLE after initiating mycophenolate mofetil for active SLE is reported. The reports of 37 women and six men  including our patient with TEN-like ACLE were also reviewed. The diagnosis of SLE or subacute cutaneous lupus erythematosus was either previously confirmed or established at the time of diagnosis of TEN-like ACLE in 41 patients. Fever was present in 59% of patients. The onset of TEN-like ACLE was either subacute (73%) or acute (27%). Thirteen cases did not clarify the nature of disease onset. The skin lesions often presented initially on sun-exposed sites (29 patients) and involved one or more mucous membranes (21 patients). A new medication may have caused the TEN-like ACLE in 67% of the patients. Systemic corticosteroids either alone or combined with hydroxychloroquine, intravenous immunoglobulin, or mycophenolate mofetil were the most commonly used treatment. Patients with TEN-like ACLE patients had an 89% survival.

Topics: Adult; Anti-Inflammatory Agents; Humans; Lupus Erythematosus, Cutaneous; Male; Mycophenolic Acid; Stevens-Johnson Syndrome

To describe the clinical features and course of 2 patients with autoimmune diseases and their experience with the Boston keratoprosthesis. To draw on general medical literature to try to better understand recurrent complications.. Retrospective review of 2 patients treated with Boston keratoprostheses. The clinical histories, examinations, and other diagnostics were reviewed. A literature review was performed.. The first patient presented with end-stage ocular disease secondary to toxic epidermal necrolysis (TENS). The second patient presented with end-stage ocular disease secondary to mucous membrane pemphigoid (MMP). Both patients underwent treatment with the Boston keratoprosthesis. Both patients suffered numerous corneal melts requiring multiple repeat implantations.. Patients with corneal blindness secondary to autoimmune disease often fare poorly with available surgical treatments. Study of existing literature on prosthetic device complications in autoimmune diseases may help uncover common mechanisms of tissue destruction to establish perioperative immunomodulatory regimens targeted to specific underlying diseases.

Topics: Adult; Alternaria; Anti-Inflammatory Agents; Antibodies, Monoclonal; Autoimmune Diseases; Blindness; Corneal Diseases; Female; Humans; Infliximab; Male; Middle Aged; Mycophenolic Acid; Mycoses; Pemphigoid, Benign Mucous Membrane; Prednisolone; Prostheses and Implants; Prosthesis Implantation; Stevens-Johnson Syndrome; Treatment Outcome; Visual Acuity

Topics: Adult; Biopsy, Needle; Dose-Response Relationship, Drug; Drug Administration Schedule; Erythema Multiforme; Follow-Up Studies; Humans; Male; Mycophenolic Acid; Recurrence; Severity of Illness Index; Stevens-Johnson Syndrome; Treatment Outcome