Urticarial Vasculitis

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Urticarial vasculitis is usually an acquired idiopathic phenomenon but may also occur in association with other disorders, such as SLE or Sjogren's syndrome (Davis et al., 1998; Sanchez et al., 1982;

Mehregan et al., 1992; O'Loughlin et al., 1978;, Provost et al., 1980). All of the patients reported with urticarial vasculitis in association with SLE have shown varying combinations of multisystem disease, and two forms of urticarial vasculitis have been described (Provost, 2004). The most common form, occurring in approximately 10% of patients with SLE, is associated with various autoantibodies (anti-dsDNA, anti-Ro/SSA, and anti-U1RNP antibodies) and, furthermore, these patients show a systemic disease, including glomerulonephritis and arthritis. Most of these patients also demonstrate a leukocytoclastic angiitis and, in some cases, a mono-nuclear vasculopathy has been described. The second form of urticarial vasculitis seen in patients with SLE is characterized by the presence of anti-C1q antibodies and hypocomplementemia. This form of urticarial vasculitis can also occur in patients without SLE and is termed hypo-complementemic urticarial vasculitis (HUVS) (Uwatoko and Mannik, 1988). Clinically, these patients frequently demonstrate arthralgias, arthritis, glomerulonephritis, angioedema, ocular inflammation (conjunctivitis, episcleritis, uveitis, etc.), and obstructive lung disease.

Urticarial vasculitis has usually a chronic course lasting for years; however, complete resolution of the skin lesions has also been described. The urtic-arial skin lesions are characterized as transient, well-circumscribed, edematous, lightly erythematous wheals, often with central clearing or a peripheral halo. However, the individual lesions usually persist for 24 h or longer. Occasionally, these lesions resolve with secondary changes, e.g. pigmentation, scaling, or purpura. With regard to symptoms, some patients complain of burning or painful lesions as opposed to pruritic lesions of classic urticaria. The typical histologic feature is a fibrinoid necrotizing vasculitis in the upper dermis, with leukocytoclasis and erythrocyte diapedesis. Direct immunofluores-cence of urticarial lesions demonstrates various combinations of immunoglobulins and complement components in the vessels walls.

3.3. Papulonodular mucinosis

Mucin deposition is generally restricted to primary mucinoses, such as lichen myxedematosus, and is rarely present in sufficient quantity to produce lesions that are clinically detectable in other diseases, such as LE (Kanda et al., 1997). Although recognized as early as 1954 by Gold (1954), only approximately 40 patients with papulonodular mucinosis associated with various forms of LE have been described in the literature (Sonntag et al., 2003; Hazan, 2004; Ortiz et al., 2004). In most cases, papulonodular mucinosis is associated with SLE, but can also be found in patients with DLE and SCLE. Interestingly, it has been indicated that papulonodular mucinosis tends to occur more frequently in males, indicating a possible role for sex-related factors, such as androgenic hormones, in the pathogenesis of this associated disease (Kanda et al., 1997). In addition, exposure to sunlight may be a further contributing factor in the pathogenesis of papulonodular mucinosis (Nagy et al., 1967; Moulin et al., 1980; Weidner and Djawari, 1982; Sonntag et al., 2003).

Clinically, papulonodular mucinosis presents with asymptomatic skin-colored papules and nodules that can be seen inside and outside of typical lesions of LE and involve mostly the trunk and upper extremities, but the face and other locations can also be affected. In some patients, papulonod-ular mucinosis predates LE, and in others it is associated with its activity, and it can even be the only cutaneous manifestation of LE during a period of time (Sonntag et al., 2003). The histologic picture of papulonodular mucinosis is dominated by abundant interstitial mucin deposition in the papillary and reticular dermis, which can be confirmed by colloidal iron staining, and there is also a mild superficial perivascular lymphocytic infiltrate. However, the characteristic alterations of LE, such as epidermal involvement and vacuolar degeneration of the dermoepidermal junction, are absent (Kanda et al., 1997; Weigand et al., 1981).

Several cutaneous mucinoses might lead to diagnostic confusion with papulonodular mu-cinosis, including lichen myxedematosus, reticular erythematosus mucinosis, and pretibial or generalized myxedema (Reed et al., 1973; Rongio-letti and Rebora, 1991, 2001). Lichen myxede-matosus most likely bears striking similarities to papulonodular mucinosis, but the clinical features differ in the form of the papules, which tend to be smaller, and the localization is most common on the face and arms.

3.4. Annular erythema

Annular erythema has been recognized as a cutaneous manifestation of Sjogren's syndrome in Asian patients and, in only one case report, it has been described in a white female (Haimowitz et al., 2000; Watanabe et al., 1997; Teramoto et al., 1989). However, in certain cases it can also represent an unusual skin manifestation within the spectrum of LE (Kuhn et al., 2000b; Ruzicka et al., 1991). Since there are common pathophysiologic mechanisms, mainly the presence of anti-Ro/SSA or anti-La/SSB antibodies, it is in some cases difficult to separate annular erythema from SCLE. However, more recent studies demonstrated that annular erythema associated with Sjogren's syndrome can clinically and histologically be differentiated from SCLE and that HLA-DRw52/-Cw3 is closely related to patients with annular erythema (Hoshino et al., 1992; Katayama et al., 1991, 1994; Miyagawa, 1994; Provost et al., 1988a; Watanabe et al., 1997).

Clinically, annular erythema is characterized by erythematous papules and centrifugal extension leading to urticarial plaques with annular elevated borders and central hyperpigmentation. In majority of patients, the erythema appears symmetrically on the face, the trunk, or the extensor aspects of the extremities, but the entire integument may also be involved. Histologic investigation shows slight epidermal changes without dyskeratotic cells or follicular plugging and reveals no changes at the dermoepidermal junction. A lymphocyte infiltration around the blood vessels and a marked edema are seen in the papillary dermis. Additional studies demonstrated prominent mucin deposition in these lesions (Kuhn et al., 2000b; Nishikawa and Provost, 1991).

4. Conclusions

Cutaneous manifestations in patients with LE are very frequent, show a great variety and can occur at any stage of the disease. A classification system has been established dividing the skin lesions associated with LE in specific and non-specific manifestations. The non-specific manifestations such as urticaria vasculitis and annular erythema, are also seen in conditions other than LE and are thus not specific for this disease. Although the specific cutaneous manifestations of this disease are considered to have a less severe course and to carry a better prognosis than the systemic form, 67-70% of patients with SCLE and 14-27% of patients with DLE have extracutaneous signs (Tebbe and Orfanos, 1997). Patients who have a more generalized involvement of the skin tend to have more systemic symptoms than those with lesions localized to the face or neck. Nevertheless, all cutaneous manifestations of LE can result in limited patient quality of life and disability from work. It has also been found that 45% of patients with CLE experience some form of vocational handicap. However, recent epidemiological data of the cutaneous manifestations of LE are not available (Jimenez et al., 2004). Therefore, it has been a long standing need for a study group including experts of different medical specialities in order to better focus on all aspects related to LE. In 2004, the 'Interdisciplinary Study Group of Lupus Erythematosus (ISGLE)' was founded with the aims (i) to receive an overview of the spectrum of clinical and laboratory features and therapeutic strategies of this disease, (ii) to develop criteria for disease activity and diagnostic guidelines for patients with LE, and (iii) to implement evidence based management strategies and standards of care for patients with the various manifestations of this disease. Further aims of ISGLE are (i) continuous standardized evaluation of the different organ systems, (ii) continuous exchange between the involved specialists on problems of their patients (e.g., pregnancy); (iii) communication of early diagnosis and treatment as well as patient education improving the quality of life and the socioeconomic status of patients with LE (http:// www.ISGLE.rheumanet.org).

5. Acknowledgements

This work was supported by Heisenberg professorship from the German Research Association (DFG) to A.K. (KU 1559/1-1).

Key points

• The clinical expression of skin involvement in lupus erythematosus (LE) is very frequent and shows a great variety.

• Gilliam (1977) initially proposed a classification system for the skin lesions that can be encountered in patients with LE and divided the cutaneous manifestations of this disease into those that are histologically specific for LE (i.e., LE-specific skin disease) and those that are not histologically specific for this disease (i.e., LE-non-specific skin disease).

• In 2003, a modified classification system of cutaneous LE (CLE) has been developed including acute CLE, subacute CLE, chronic CLE, and the intermittent subtype of CLE, ICLE.

• Non-specific cutaneous manifestations of LE which can also occur in association with other disorders include non-scarring alopecia, annular erythema, calcinosis cutis, papulonodular mucinosis, rheumatoid nodules, and sclerodactyly. Furthermore, several skin diseases can also be found in the context of LE such as acanthosis nigricans, anetoderma, cutaneous vascular diseases, e.g., Raynaud's phenomenon and urticaria vasculitis, erythema multiforme, bullous autoimmune diseases, lichen planus, and porphyria cutanea tarda.


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Handbook of Systemic Autoimmune Diseases, Volume 5 The Skin in Systemic Autoimmune Diseases

Piercarlo Sarzi-Puttini, Andrea Doria, Giampiero Girolomoni and Annegret Kuhn, editors

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