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55
1995) in their elegantly executed efforts to better document the relationships that exist
between the cutaneous and systemic manifestations of LE in Japanese patients.
Further efforts have been made to simplify the classification of LE by developing
classification trees through the methods of recursive partitioning (Edworthy et al.
1988). In addition, much work has been expanded to subclassify the cutaneous manifestations of this disease into more uniform patient groups (Costner et al. 2003, Sontheimer and Provost 1996), and a variety of subclassification criteria have been used:
clinical features, laboratory findings, histopathologic and immunohistologic patterns, genetic associations, and, more recently, phototesting results. Provocative phototesting has been crucial in further characterizing a highly photosensitive form of
CLE, namely, LE tumidus (LET) (Kuhn et al. 2000). Although LET was first mentioned
by Hoffmann in 1909 (Hoffmann 1909), and some years later by Gougerot and Bournier
(Gougerot and Burnier 1930), this subtype had since been somewhat forgotten or
rarely described in the literature. In 1990, Goerz et al. (Goerz et al. 1990) emphasized
for the first time the extreme photosensitivity as a major characteristic of LET, and
additional studies showed that these patients were more photosensitive than those
with other forms of CLE (Kuhn et al. 2001a). Furthermore, in our experience the prognosis in patients with LET is generally more favorable than in those with other forms
of CLE and, interestingly, LET lesions can disappear spontaneously within days or
weeks, even if the disease recurs chronically in these patients. Nevertheless, characteristic LET lesions can also occur in patients with SLE (Jolly et al. 2004). Recent
studies of more than 60 patients with LET further confirmed that this subtype of CLE
has so many characteristic features that it should be considered as a separate entity
and differentiated from ACLE, SCLE, and CCLE (Kuhn et al. 2000, 2001b, 2002a, b,
2003). Meanwhile, there is no doubt about LET being a separate entity, and further
case reports of this disease followed from groups other than our own (AlexiadesArmenakas et al. 2003, Hsu et al. 2002, Pacheco et al. 2002). Therefore, based on the
recent published data on LET we developed a modified classification system including
LET as an intermittent subtype of CLE (ICLE) (Kuhn 2003). In addition, bullous skin
lesions associated with different forms of LE (BLE) have also been added to the new
Table 5.1. Dsseldorf Classification of Cutaneous Lupus Erythematosus 2003
56
Dsseldorf classification of CLE 2003 (Table 5.1). The various types of vesicular and
bullous skin lesions that can occur in patients with LE have been divided into those
that show or do not show LE-specific histopathology (Sontheimer 1997, Yell and
Wojnarowska 1997). Furthermore, a number of primarily blistering diseases, such as
bullous pemphigoid, dermatitis herpetiformis, and pemphigus vulgaris, has been
reported in relationship to LE. Whether these bullous skin disorders are the result of
the LE autoimmune disease process or develop as a mere chance occurence in
patients who also have LE is not clear.
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