Systemic lupus erythematosus with multiple dermatofibromas – Drexel – May 16, 2016

Presented by: Chloe Etzler, MD, Jeanyoung Kim, MD, and Herbert Allen, MD

Drexel University College of Medicine

May 16, 2016

Approximately 80% of patients with systemic lupus erythematosus (SLE) have cutaneous manifestations. They are often the initial presenting complaint. Cutaneous findings include calcinosis cutis, telangiectasias, malar rash, discoid lesions, photosensitivity, and oral ulcers. Patients with skin involvement are more likely to have systemic findings involving the immunologic, renal, neurologic or rheumatologic systems. Fever and lymphadenopathy may also accompany the onset of SLE along with serositis.

Multiple eruptive dermatofibromas (MEDF) have been described in association with SLE. Dermatofibromas, also sometimes referred to as histiocytomas, are common benign tumors. These firm, hyperpigmented papules are most often found on the legs of women. Generally, these are solitary and not associated with any internal disease. On histology, the dermatofibromas of MEDF have no distinguishing features to separate them from solitary lesions. A well circumscribed dermal tumor of spindle cells is classically seen. Collagen trapping at the periphery is typical.

Multiple eruptive dermatofibromas were originally defined as greater than 15 lesions. This number was later revised to include the presence of five to eight, which arise in the course of six months. Since the original description of this condition in a female patient with 61 lesions, numerous associations have been documented. Only a minority of MEDF occur in healthy patients. MEDF has been seen in patients with autoimmune diseases including SLE, dermatomyositis, Sjӧgren’s syndrome, pemphigus vulgaris, and myasthenia gravis. It has also been associated with conditions of impaired immunity including HIV and a case of severe atopic dermatitis treated with long term topical corticosteroids. Other associations include pregnancy, hematologic malignancies, diabetes mellitus, ulcerative colitis, and mycosis fungoides. Interestingly, patients with both SLE and Sjӧgren’s syndrome, as well as SLE and HIV, have been described with MEDF. In addition to these systemic diseases, several immunosuppressant medications have been linked to MEDF including glucocorticoids, azathioprine, methotrexate, anti-TNF-α therapy, and cyclophosphamide.

The etiology of MEDF is unknown. Dermatofibromas are generally believed to result from minor trauma, such as an insect bite. Subsequent fibroblast activation or inflammatory response may cause the development of dermatofibromas. MEDF has been seen arising both prior to and during the treatment of the underlying disease states. This suggests that MEDF may be triggered by the connective tissue disease itself as well as by the immunosuppressive agents. One example is a patient with SLE and Sjӧgren’s syndrome who developed MEDF prior to institution of therapy. In contrast, another patient has been described as acquiring MEDF during a period of SLE remission, thus suggesting that his continued immunosuppressive regimen of prednisolone, diaphenylsulfone, and cyclosporine, may have been the cause. In clinical practice, when encountering MEDF, it would be prudent to evaluate the patient for signs of autoimmune disorders, connective tissue disease, HIV, or other systemic illness.

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