Life-threatening manifestation of cutaneous leukocytoclastic vasculitis

Cutaneous leukocytoclastic vasculitis (LCV) is a form of small-vessel vasculitis with a predilection for skin involvement. In about half of the cases the condition has an idiopathic origin, whereas the remaining cases develop secondarily to various causes, such as infections, connective tissue diseases, malignancies, and drugs [1]. Although LCV is generally considered as self-limiting and has a good overall prognosis [2], several studies have described less favourable, chronic, and/or recurrent course of the disease [3, 4]. Hence, therapeutic options depend on the severity of disease, and range from antihistamines, topical and/or oral corticosteroids (CS), to immunosuppressive drugs [4, 5]. Herein, we report the case of a 46-year-old man with a history of multiple, deep skin ulcerations over the trunk and extremities, later confirmed as LCV. The patient had been treated with a combination of systemic CS and infusions of intravenous immunoglobulins (IVIG) within the 29-month period of follow-up, which gradually lead to significant, although to date not complete, reduction of skin lesions, and remarkably improved the quality of the patient’s life.
A 46-year-old Caucasian male with no significant previous medical history was admitted to the Rheumatology Department in December 2016, presenting with extensive skin ulcerations of the trunk and the extremities in the course of newly diagnosed LCV. The first symptoms of the disease developed about 6 months earlier and included recurrent swelling of the right leg, accompanied by purpuric rash on the lower extremities and subsequent formation of non-healing skin ulcerations. The patient’s history of constitutional symptoms, preceding infection, and drug intake was negative. The laboratory results revealed raised inflammatory markers (erythrocyte sedimentation rate (ESR) – 52 mm/h, C-reactive protein (CRP) – 99.5 mg/l), thrombocytosis, anaemia of chronic diseases (normocytic anaemia with low serum iron level, decreased TIBC, increased concentration of serum ferritin, normal RDW, and normal reticulocyte count), and decreased serum albumin and proteinuria. The serological findings, including antineutrophil cytoplasmic antibodies (ANCA), antinuclear antibodies (ANA), antiphospholipid antibodies (anticardiolipin and antib2GP1 antibodies), were negative. Physical examination revealed numerous, extensive skin ulcerations with focal necrosis over the trunk, and the upper and lower extremities, including the hands...


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