Vasculitis
Vasculitis is defined as an inflammatory cell infiltration and the destruction of blood vessels identified upon histological examination. Cutaneous manifestations may be the first and only manifestation of the disease or be a part of a systemic condition.
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Vasculitis Nicolas Kluger
20.1
Contents 20.1
Diagnosis of Cutaneous Vasculitis Is Made on Histology ..................................
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20.2
Pitfalls ..........................................................
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20.3
Clinical Pathologic Correlation .................
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Clinical Manifestations...............................
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Classification ...............................................
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20.6
Approach to the Diagnosis of Cutaneous Vasculitis ..............................
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Management of Cutaneous Vasculitis ......................................................
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References ...............................................................
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20.7
N. Kluger Departments of Dermatology, Allergology and Venereology, Institute of Clinical Medicine, University of Helsinki and Skin and Allergies Hospital, Helsinki University Central Hospital, Helsinki, Finland e-mail: [email protected]
Diagnosis of Cutaneous Vasculitis Is Made on Histology
Physical cutaneous signs of vasculitis are wide and nonspecific. Cutaneous vasculitis (CV) affects the skin with varying intensity, depth, and distribution. Even though a certain number of syndromes have been described, a patient may present with symptoms that overlap with another clinical diagnosis making a diagnosis “at first sight” impossible. Most of all, vasculitis has a histopathologic definition; therefore, its confirmation comes only from the microscopic examination of the lesion [1–5]. The diagnosis of CV is made by microscopic examination of hematoxylin-eosin-stained biopsies. A list of criteria allows a trained pathologist to diagnose and distinguish an active vasculitis from chronic and healed lesions of vasculitis and changes that are adjacent to vasculitis and may help to define a subtype or the etiology of the CV. Inflammatory infiltrates within and around the vessel walls associated by intramural and/or intraluminal fibrin deposition (fibrinoid necrosis) confirm the diagnosis of vasculitis. Some changes are suggestive of active vasculitis such as red blood cell extravasation, perivascular nuclear dust (leukocytoclasia), eccrine gland necrosis, ulceration, and necrosis/infarction. In the absence of fibrinoid necrosis, the diagnosis of CV becomes more difficult. Lamination of the adventitia, media, and/or intima; perivascular nuclear dust
L. Téot et al. (eds.), Skin Necrosis, DOI 10.1007/978-3-7091-1241-0_20, © Springer-Verlag Wien 2015
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N. Kluger
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(leukocytoclasia) without fibrinoid necrosis; loss of the elastic lamina with acellular scar tissue; or subendothelial intramuscular and/or adventitial inflammatory cells in large vessels are all other indications for vessel wall damages [1–5]. A direct immunofluorescence examination (DIF) is also mandatory in case of CV. It does not confirm the diagnosis of CV but allows to determine one or another diagnosis. • The absence of immune complex is in favor for pauci-immune vasculitis: Wegener granulomatosis (WG), Churg-Strauss syndrome (CSS), and microscopic polyan
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