Vasculitis is a group of uncommon conditions characterized by inflammation within the walls of blood vessels (arteries, veins, and capillaries). This inflammation causes vessel walls to thicken, narrow, or scar. Restricted blood flow can damage the tissues and organs supplied by those vessels. Because blood vessels extend throughout the entire body, vasculitis is a systemic condition that can affect nearly any organ. Symptoms depend largely on the size and location of the blood vessels involved.
Recognizing Symptoms on the Face
The facial area, head, and neck can show distinct outward signs of blood vessel inflammation. One recognizable skin manifestation is palpable purpura, which appears as raised, non-blanching red or purple spots. These lesions result from damaged small blood vessels leaking blood into the surrounding tissue.
Other rashes can mimic common skin conditions. Urticaria-like lesions look similar to hives but persist for more than 24 hours and are often painful or burning. Swelling of the face and neck, known as angioedema, may also occur. This swelling sometimes appears as a puffy discoloration around the eyes, referred to as periorbital ecchymosis.
More severe forms of vasculitis can lead to painful, slow-healing nodules or ulcers on the face. Inflammation affecting medium-sized vessels can cause livedo reticularis, a mottled, lace-like pattern of reddish-blue discoloration. This pattern is caused by disrupted blood flow in the capillaries just beneath the skin’s surface.
When vasculitis involves the upper respiratory tract, such as in Granulomatosis with Polyangiitis (GPA), facial symptoms extend beyond the skin. Persistent inflammation of the sinuses and nasal passages can lead to recurrent nosebleeds and crusting. Over time, destruction of the nasal septum cartilage can cause the bridge of the nose to collapse, resulting in a saddle nose deformity. Giant Cell Arteritis (GCA) can involve the temporal arteries, causing scalp tenderness and, rarely, tissue death (necrosis) of the scalp or tongue.
Underlying Causes and Associated Conditions
Vasculitis is an autoimmune process where the immune system mistakenly targets blood vessel walls. It can occur on its own (primary vasculitis) or develop secondary to an underlying illness or trigger. The specific type of vasculitis determines which vessels are affected and which facial structures show symptoms.
Granulomatosis with Polyangiitis (GPA) is a small-to-medium vessel vasculitis that frequently involves the upper respiratory tract, causing severe facial and sinus-related symptoms. Giant Cell Arteritis (GCA) is a large vessel vasculitis that predominantly affects the arteries of the head and neck, including the temporal artery. GCA often presents with severe headache and jaw pain alongside facial skin changes. Polyarteritis Nodosa (PAN), a medium vessel vasculitis, can also cause nodules and ulcers on the face.
Possible triggers for secondary vasculitis include certain infections (such as hepatitis B and C) or chronic inflammatory autoimmune disorders (like systemic lupus erythematosus or rheumatoid arthritis). Certain medications, including some antibiotics and nonsteroidal anti-inflammatory drugs, are potential causes of temporary hypersensitivity vasculitis. The inflammation is often driven by the deposition of immune complexes (clumps of antibodies and antigens) or by autoantibodies like antineutrophil cytoplasmic antibodies (ANCA).
Confirming the Diagnosis
Diagnosis begins with a detailed medical history and a thorough physical examination to assess the facial and systemic symptoms. Since many conditions mimic vasculitis, laboratory tests check for signs of widespread inflammation. These tests include measuring inflammatory markers such as the Erythrocyte Sedimentation Rate (ESR) and C-reactive protein (CRP), which are often elevated in active disease.
A blood test for Antineutrophil Cytoplasmic Antibodies (ANCA) is important when GPA is suspected, as these antibodies are strongly associated with small vessel vasculitis. However, blood tests alone cannot confirm the diagnosis. The definitive method for diagnosing vasculitis is a tissue biopsy from an affected area, which is considered the gold standard.
When facial skin lesions are present, a punch biopsy of the affected skin is performed. This allows a pathologist to examine the blood vessel walls under a microscope for inflammation. For suspected GCA, a temporal artery biopsy is often required. Imaging studies, such as computed tomography (CT) or magnetic resonance imaging (MRI), assess deeper facial structures, like the sinuses and orbits, to determine the full extent of the disease and plan treatment.
Treatment Approaches
Treatment is tailored to the specific type and severity of the disease. Primary goals are to reduce inflammation and suppress the overactive immune system. Initial treatment often involves high-dose corticosteroids, such as prednisone, which rapidly control acute inflammation. This phase is known as remission induction therapy.
For severe, organ- or tissue-threatening disease, high-potency immunosuppressive drugs are often added to corticosteroids. Medications like cyclophosphamide or rituximab are commonly used during the induction phase to quickly control systemic inflammation and prevent irreversible damage, such as nasal structure collapse. Once the disease is stable and in remission, the focus shifts to maintenance therapy, where the corticosteroid dose is slowly reduced.
Maintenance therapy involves less toxic immunosuppressants, such as methotrexate or azathioprine, to keep the disease in check long-term. Managing localized facial symptoms is important to prevent scarring or permanent tissue loss. This may involve specific wound care for ulcers and nodules, or surgical consultation for structural issues like saddle nose deformity once disease activity is controlled.