Leukocytoclastic Vasculitis: An Early Skin Biopsy Makes a Difference

Abstract

Leukocytoclastic vasculitis (LCV) is an uncommon condition with a broad differential diagnosis. Although the clinical history, physical examination, and laboratory workup are pivotal when formulating a differential diagnosis of LCV, a skin biopsy is required in most cases to elucidate the cause. The diagnostic yield of a skin biopsy increases within the first 24 to 48 hours of the lesion onset indicating the importance of obtaining a prompt skin sample. We present the case of a 60-year-old man who presented to the emergency department with a three-day history of fevers, headaches, and a painful skin rash. He endorsed rhinorrhea and sore throat a week ago. Physical examination was notable for an erythematous papular rash with palpable violaceous purpura located mainly at the distal right leg and thigh. He also complained of painful bilateral hand edema. His complete blood count and chemistries were unremarkable. His C-reactive protein was 147 mg/L (normal value <8 mg/L), and sedimentation rate was 51 mm (normal value <15 mm). Immunoglobulin A was 509 mg/dL (normal value 82-460 mg/dL). Further workup including viral hepatitis serologies, antinuclear antibodies, complements, antineutrophil cytoplasmic antibodies, cryoglobulins, rheumatoid factor, and blood cultures yielded negative results. Therefore, it was believed that his rash was likely associated with his recent upper respiratory infection. A skin biopsy done on the first day of admission was positive for LCV without immunoglobulin A deposition. He was managed with prednisone and anti-inflammatory medications with improvement of his rash.

Leukocytoclastic Vasculitis Secondary to Anti-Tumor Necrosis Factor Therapy in Inflammatory Bowel Diseases: A Multicenter Retrospective Cohort Study

“…In all five patients, the results of detailed laboratory investigations for inflammatory markers, autoimmune markers, immunoglobulin (IgG, IgA, and IgG4) and complement (C3 and C4) serum levels, viral serology, infectious work-up, urinalysis, creatinine, and liver function were unremarkable. Although a patient's clinical history and a physical examination and laboratory work-up are important for a differential diagnosis of LCV, a histology is essential to confirm a diagnosis of vasculitis and to avoid a delayed or erroneous diagnosis that could lead to improper management [19,28]. The differential diagnosis includes systemic primary small-vessel vasculitis (such as ANCA-associated vasculitis, cryoglobulinemic vasculitis, and IgA vasculitis), thrombocytopenic purpura, infections, and rheumatic and autoimmune diseases, such as rheumatoid arthritis, Sjogren's syndrome, Behçet's disease, systemic lupus erythematosus, and relapsing polychondritis [7].…”

A Case of Adult-Onset IgA Vasculitis in a Cirrhotic Patient

“…Some of the expected biopsy findings include fibrinoid necrosis, inflammatory cells infiltrating the adventitia and media, and extravasation of red blood cells causing purpura. Multiple authors agree that a biopsy should be performed between 24 and 48 hours of lesion onset to increase the diagnostic yield [ 3 ]. Ideally, a representative lesion should be sampled when it is relatively “fresh” yet well-established [ 4 ].…”

Localized Forms of Vasculitis

“…It should preferably be performed within the first 48 h after the appearance of skin lesions [10]. If more time has passed, the biopsy should be directed at lesions with 24 to 48 h old [5,24]. Typical histologic findings include the presence of vascular and perivascular infiltration, predominantly of neutrophils, particularly affecting the superficial dermal plexus; formation of nuclear dust (leukocytoclasis); extravasated erythrocytes; and fibrinoid necrosis of the vessel walls (Fig.…”