A resistant case of fulminant lupus profundus.

Lupus profundus (LP) is an uncommon but distinct subset of systemic lupus erythematosus (SLE). The course of LP is usually benign. Uncommonly, ulcerations may be seen with concomitant discoid lupus. We describe a rare case of aggressive LP resistant to conventional treatment. A 44-year-old male with a history of SLE diagnosed in 1994, hypertension and alcohol abuse presented in 2002 with a new onset of cutaneous lesions. These manifested as multiple nodules, papules and ulcerations with surrounding erythema distributed bilaterally on elbows and hands. Laboratory data revealed antinuclear antibody 1:640, positive serologies for ribonucleoprotein and Rho, as well as decreased levels of complement C3. Biopsy of the lesions revealed hyaline necrosis of adipose tissue and lymphocytic infiltrate compatible with panniculitis. The patient was initially treated with corticosteroids and hydroxychloroquine without resolution of the lesions. Subsequently, a combination of corticosteroids and azathioprine was administered to which he demonstrated no response. Despite treatment, the lesions progressed to develop purulent drainage. Methotrexate was not initiated due to alcohol use. IV pulse cyclophosphamide was initiated, however the patient was hospitalized with worsening symptoms soon after. On admission, he was noted to have multiple punctate ulcerations and tender nodular subcutaneous lesions in various stages of healing involving more than 30% of the body surface area. Notably, the right ring fingertip appeared infarcted suggesting concomitant vasculitis. The patient continued to deteriorate, and the course was complicated by staphylococcal sepsis that resulted in his death. The reported incidence of LP in association with SLE is 2%. Previous studies concluded that LP cases who fulfill criteria for SLE tend to have a milder systemic involvement. This case illustrates that although usually benign, fulminant LP resistant to conventional treatment may occur and although uncommon, may occur in males.

Villa Del Carmen, MD, Andres C. Marte-Grau, MD, and John Pendleton, MD. University of Virginia, Roanoke-Salem Program, Roanoke, VA.