Gibson L E
Department of Dermatology, Mayo Medical School, Rochester, Minnesota.
Dermatol Clin. 1990 Apr;8(2):335-45.
Wegener's granulomatosis, lymphomatoid granulomatosis, and Churg-Strauss granulomatosis may all have cutaneous involvement. The morphology of cutaneous lesions in these disorders varies from macular erythema to frank gangrenous ulceration. Most often lesions are located on the extremities; however, truncal or facial involvement has been reported, the latter especially in Wegener's granulomatosis. A common histologic finding in these cutaneous lesions is necrotizing vasculitis. However, it is also possible to see Churg-Strauss extravascular granulomas and even periarteritis. Cutaneous involvement with these three forms of systemic granulomatosis generally parallels the systemic course. The treatment for the cutaneous lesions is dictated by the treatment for the systemic vasculitis. It is important to recognize that the cutaneous extravascular granuloma and cutaneous granulomatous vasculitis can be associated with other disorders in addition to systemic granulomatosis. These disorders include most importantly lymphoproliferative diseases, inflammatory disorders such as arthritis, autoimmune diseases, and other inflammatory disorders such as sarcoidosis. Cutaneous involvement with giant cell or temporal arteritis is not common, but ulcerative temporal-parietal scalp lesions are distinctive. Although not common in the United States, Takayasu's arteritis may have several cutaneous manifestations, including erythema nodosum-like lesions. Granulomatous vasculitides have a myriad of cutaneous manifestations. Knowledge of these manifestations may allow for prompt diagnosis in many cases and increased surveillance in other cases for associated systemic illnesses.
韦格纳肉芽肿、淋巴瘤样肉芽肿和变应性肉芽肿性血管炎均可能累及皮肤。这些疾病中皮肤损害的形态各异,从斑疹性红斑到明显的坏疽性溃疡。皮损最常位于四肢;然而,也有躯干或面部受累的报道,后者在韦格纳肉芽肿中尤为常见。这些皮肤损害常见的组织学表现是坏死性血管炎。不过,也可见到变应性肉芽肿性血管炎的血管外肉芽肿,甚至动脉周围炎。这三种系统性肉芽肿病的皮肤受累情况通常与全身病程平行。皮肤损害的治疗取决于系统性血管炎的治疗。必须认识到,皮肤血管外肉芽肿和皮肤肉芽肿性血管炎除了与系统性肉芽肿病相关外,还可能与其他疾病有关。这些疾病最重要的包括淋巴增殖性疾病、炎症性疾病如关节炎、自身免疫性疾病以及其他炎症性疾病如结节病。巨细胞动脉炎或颞动脉炎累及皮肤并不常见,但颞顶头皮溃疡性损害具有特征性。虽然高安动脉炎在美国并不常见,但它可能有多种皮肤表现,包括结节红斑样损害。肉芽肿性血管炎有众多的皮肤表现。了解这些表现可能在许多情况下有助于及时诊断,并在其他情况下加强对相关系统性疾病的监测。