Wegeners granulomatosis

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WG is characterized by necrotizing inflammatory lesions of the respiratory tract, usually accompanied by glomerulonephritis and/or systemic vasculitis. Klinger (1931) was the first to describe the disease, followed 5 years later by Wegener (1936). Detection

Table 3

Cutaneous manifestations of Churg-Strauss syndrome

References No. of patients No. with skin Cutaneous manifestations, when detailed

Table 3

Cutaneous manifestations of Churg-Strauss syndrome

References No. of patients No. with skin Cutaneous manifestations, when detailed

involvement (%)

Abu-Shakra et al., 1994




Lanham et al., 1984



Nodules = 2; purpura = 9; urticarial macules


Haas et al., 2001



Nodules = 6; purpura = 5; urticarial lesions =

3; other(s) = 6

Chumbley et al., 1977



Nodules = 8, necrosis = 4

Solans et al., 2001



Purpura = 16; maculopapular rash = 10; digit

necroses = 3;

nodules = 2

Davis et al., 1997



Skin lesions as presenting sign = 5

Guillevin et al., 1999a



Purpura = 30; nodules = 19; urticarial lesions =

8; livedo = 6;

infiltrated papules = 2

infiltrated papules = 2

of C-ANCA, with anti-PR3 specificity is an important clue to the diagnosis, as is paranasal sinus biopsy, which is diagnostic for over 50% of the cases, whereas the yield from nasal or laryngeal mucosa biopsy is less than 20% (Devaney et al., 1990).

Skin lesions occur in 10-50% of the patients (see Table 4), at some time during the course of the disease (Anderson et al., 1992; Brandwein et al., 1983; Daoud et al., 1994; de Groot et al., 2001a; Fauci et al., 1983; Guillevin et al., 1997; Hoffman et al., 1992; Koldingsnes and Nossent, 2003; Lie, 1997; Reinhold-Keller et al., 2000; Stone, 2003; Walton, 1958). They may be present at disease onset in about 10% of the patients and, exceptionally as the presenting symptom (Frances et al., 1994; Hoffman et al., 1992). Palpable purpura of the lower extremities is undoubtedly the most frequent cutaneous manifestation. Necrotic papules on the extensor surfaces of the limbs are less frequent but more suggestive of WG. Occasionally, they can resemble erythema elevatum diutinum and may be associated with IgA paraproteinemia. Nodules are frequent, mainly on the limbs. Extensive and painful cutaneous ulcerations may precede by several weeks to several years other systemic manifestations. Ulcers are sometimes described as 'pyoderma gangrenosum-like lesions', especially when occurring after even minor trauma to painful nodules or pustules. However, they usually lack the typical raised, tender, outlined border of pyoderma gangrenosum. Sometimes multiple, they are localized on the limbs, trunk, face (pre-auricular area), breasts and perineum. Lesions on the breasts may mimic adenocarcinoma with possible nipple retraction and galactorrhea (Trueb et al., 1999). Digital gangrene has occasionally been reported (Handa and Wali, 1996). Florid xanthelasma is associated with longstanding granulomatous orbital and periorbital infiltration (Frances et al., 1994; Tullo et al., 1995). In contrast to PAN, livedo reticularis is unusual in WG.

Mucosal manifestations might also be suggestive of WG. Buccal lesions are undoubtedly frequent, often reported as one of the ear, nose and throat manifestations of WG, present in 10-50% of the patients (D'Cruz et al., 1989; Frances et al., 1994). Their numbers and localizations vary widely, and, unlike recurrent aphthae, they are persistent. Hyperplastic gingivitis and gums are rare, but with some well-documented case reports (Patten and Tomecki, 1993). The gingiva is generally described as granular and red to purple with many petechiae. The entire gingiva and periodontium may be involved resulting in tooth mobility and loss. Major but incomplete regression can be obtained with empiric antimicrobial therapy. Genital ulcers are

Table 4

Cutaneous manifestations of Wegener's granulomatosis

Reference No. of patients No. with skin Cutaneous manifestations, when detailed involvement (%)

Guillevin et al., 1997 50 15(30)

Walton, 1958 56 26(47)

Koldingsnes and 56 19(34) Nossent, 2003

Frances et al., 1994 75 35(47)

Reinhold-Keller et al., 155 51(33) 2000

Hoffman et al., 1992 158 73(46)

Lie, 1997 216 26(12)

Anderson et al., 1992 265 66(25)

Purpura = 26; nodules = 6; ulcers = 5; necrotic papules = 5; pustules = 2; palpebral xanthoma = 2; digital necrosis = 1; livedo reticularis = 1 Present at diagnosis = 11

Present at diagnosis = 32; during the whole course of the disease = 51

Present at diagnosis = 20; during the whole course of the disease = 73

Purpura = 31; nodules = 18; ulcers = 6; gangrene = 1

uncommon although penile necrosis has previously been described (Matsuda et al., 1976).

Histologically, purpuric papules correspond to leukocytoclastic vasculitis of small vessels; necrotic and purpuric lesions can be a consequence of nec-rotizing vasculitis of superficial and/or deep dermal and subcutaneous vessels. Other lesions are more frequently associated with granulomatous inflammation. Papules or papulonecrotic lesions are the histologic counterparts of leukocytoclastic or granulomatous vasculitis of small vessels, or to ex-travascular granuloma. Nodules coincide with necrotizing or granulomatous vasculitis of medium-sized arterioles, or to extravascular granuloma (Barksdale et al., 1995). All these lesions may evolve to ulceration with a secondary mixed inflammatory pattern. Histopathologic findings of oral ulcerations are often non-specific, showing mixed areas of acute and chronic inflammation, or granulomatous infiltration in some cases (Frances et al., 1994; Patten and Tomecki, 1993). Chronic histiocytic inflammation with inconstant vasculitis, necrosis and giant cells may be seen in gingival hyperplasia. Pseudo-epitheliomatous hyperplasia and microabscesses with neutrophils and eosinophils are sometimes encountered (Handlers et al., 1985).

Regardless of the type of clinical and histolog-ical skin lesions, all of them, except xanthelasma, are usually associated with active systemic disease. They disappear in a few weeks or months after the onset of treatment, but reappear in about 50% of relapses. Skin lesions were significantly associated with articular and/or renal involvement in one series of dermatologic patients (Frances et al., 1994), and with peripheral neuropathy in another (de Groot et al., 2001b). Subacute forms of WG, limited to the skin have been individualized (Carrington and Liebow, 1966; D'Cruz et al., 1989). In our experience, the most frequent lesions in skin-limited WG are nodules, with granulo-matous infiltration or granulomatous vasculitis found during histologic examination.

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