A case suggesting lymphocytic vasculitis as a presenting sign of early undifferentiated connective tissue disease.
A previously healthy 14-year-old Korean male presented with striking hemorrhagic acral livedo associated with anti-ribonucleoprotein antibodies. His skin biopsy revealed a striking lymphocytic vascular reaction with mild superficial perivascular lymphocytic infiltrates, perivascular extravasation of red blood cells, and leukocytoclastic debris. Lymphocytic vasculitis (LV) is defined in different ways by different authors. The above biopsy findings do not fulfill the criteria for LV of all authors. Chronic lymphocyte-mediated endothelial cell injury may play an important role in the pathogenesis of collagen vascular disease. Cell-mediated cytotoxicity against endothelial cells or other vessel wall components is thought to be the possible pathologic mechanism of LV. We present a patient with early undifferentiated connective tissue disease (EUCTD) with hemorrhagic acral livedo, who finally developed systemic lupus erythematosus. Even though the histopathologic findings do not meet the most rigorous definition of LV, LV may a presenting sign of EUCTD. The purpose of this report is to suggest reconsideration of the diagnostic criteria of lymphocytic vasculitis.
D000293:Adolescent; D003240:Connective Tissue Diseases / Q000473:pathology*; D003937:Diagnosis, Differential; D006801:Humans; D008208:Lymphatic System / Q000473:pathology*; D008214:Lymphocytes / Q000473:pathology*; D008297:Male; D017445:Skin Diseases, Vascular / Q000473:pathology*; D014657:Vasculitis / Q000473:pathology*