Dermatology - Wegener Granulomatosis

Dermatology - Wegener Granulomatosis
Wegener granulomatosis is a type of vasculitis that affects the entire body. It is characterized by the presence of necrotizing granulomas in the upper respiratory tract and lungs, inflammation of both arteries and veins, and inflammation of the glomeruli.

The presence of serous otitis media and erythematous swelling of the external ear may indicate eustachian tube obstruction, which can cause pain. Gingival hyperplasia, conjunctivitis, episcleritis, scleritis, granulomatous sclerouveitis, ciliary vascular vasculitis, and retroorbital mass lesion with proptosis are potential manifestations of this condition. There may be the presence of cranial neuritis, mononeuritis multiplex, and cerebral vasculitis. Pulmonary infiltrates may be seen. In cases of advanced or chronic illness, individuals may exhibit symptoms such as renal failure, elevated body temperature, pain in the paranasal sinuses, discharge from the nose that is either filled with pus or blood, coughing, coughing up blood, difficulty breathing, and discomfort in the chest.

The skin signs include symptoms of hypersensitivity vasculitis, nodulo-ulcerative lesions, and oral/nasal ulcerations. Ulcers that have irregular, undermined edges are most commonly similar in appearance to pyoderma gangrenosum. Papules, vesicles, and palpable purpura manifest similarly to hypersensitivity (necrotizing) vasculitis, whereas subcutaneous nodules, plaques, and noduloulcerative lesions are also observed. The initial manifestation of this condition is frequently oral ulcerations, which may occur alone or in conjunction with nasal mucosal ulceration, crusting, blood clots, nasal septum perforation, and saddle-nose deformity.

The diagnosis is made based on clinical and laboratory results. The blood tests indicate the presence of mild anemia and an abnormal increase in white blood cells, with or without an abnormal increase in platelets. The eosinophil sedimentation rate is significantly increased. Antineutrophil cytoplasmic autoantibodies (c-ANCA) are seromarkers that indicate the presence of certain diseases, and the levels of these antibodies are directly related to the severity of the condition. There is evidence of IgA hypergammaglobulinemia. The urinalysis reveals the presence of proteinuria, hematuria, and red blood cell casts.

Administering cyclophosphamide and prednisone therapy results in a 90% success rate in achieving long-term remission. Untreated, the illness frequently results in death.