Elsevier

Autoimmunity Reviews

Volume 8, Issue 6, May 2009, Pages 441-448
Autoimmunity Reviews

The 100th anniversary of lupus erythematosus tumidus☆

https://doi.org/10.1016/j.autrev.2008.12.010Get rights and content

Abstract

In 1909, the term “lupus erythematodes tumidus” was first introduced by the German Dermatologist E. Hoffmann. The next case reports of lupus erythematosus tumidus (LET) were not described until 1930, and in the following years, only a few further cases were reported. This might have been due to the fact that authors have not considered LET as a separate entity different from other variants of cutaneous lupus erythematosus (CLE), and it is likely that skin lesions described under different designations represent the same disease entity. Therefore, LET has been underestimated and neglected in the literature and has been characterized by clinical, histopathological, and immunohistochemical features only in recent years. In particular, phototesting has been crucial in defining LET as a very photosensitive entity of CLE. Up to now, more than 40 reports of LET have been published demonstrating that the course and prognosis of LET are generally more favorable than in other subtypes of CLE. A new classification system, including LET as the intermittent subtype of CLE (ICLE) has been suggested. On the occasion of the 100th anniversary of the first description of LET, we have reviewed the literature and provide here an overview on the different aspects of the disease.

Section snippets

History

The term “lupus erythematodes tumidus” was first used by E. Hoffmann in 1909 [1] at a meeting of the Berlin Dermatological Society. Two patients presented with single round-shaped erythematous, elevated, tumorous lesions in the face without or with minimal scaling that vanished partly under pressure. Several years later (1930–1932) Gougerot and Burnier [2], [3], [4], [5] reported five additional patients and described similar clinical features, such as erythematous, indurated, nonscarring

Epidemiology

LET has been reported in women and men, which seem to be equally affected. In some studies a slight male predominance (22 males versus 18 females) was seen [15], whereas others reported a female:male ratio of 1:1 [16] or a slight female predominance (eight females versus seven males) [17]. Most case reports of LET in the literature are published by European groups indicating that many more patients are seen in the Caucasian population [18], however, no data with regard to the prevalence and

Clinical features

Clinically, LET is characterized by indurated, succulent, urticaria-like, single or multiple plaques with a bright reddish or violaceous, smooth surface on sun-exposed areas, such as the face, upper back, V-area of the neck, extensor aspects of the arms, and shoulders. The skin lesions mostly spare the knuckles, inner aspect of the arms, and axillae [15]. A patient with LET following the lines of Blaschko has been reported [22], and recently, one single case report demonstrated the rare

Photosensitivity

It has long been suggested that LET is characterized by a remarkable photosensitivity [36]. Provocative phototesting according to a standardized protocol revealed that patients with LET are more photosensitive than those with other forms of CLE. In our study from 2001 [37], characteristic skin lesions were induced by UV radiation in 43 (72%) of the 60 patients; 30 patients (50%) reacted to UVA and 29 (48%) to UVB irradiation. Combined UVA and UVB irradiation has been used in 30 patients; 19 of

Histology and direct immunofluorescence

Histological analysis of skin lesions is necessary to confirm the diagnosis of LET, and, therefore, it represents one of the major criteria of this disease [43]. The most frequent features in biopsy specimens from LET lesions are a fairly well-circumscribed lymphocytic dermal infiltrate in a perivascular and periadnexal pattern and abundant interstitial mucin deposition. The dermis shows edema in its upper part; however, in contrast to other subtypes of CLE, epidermal changes, such as atrophy

Immunohistochemistry

Immunohistological studies helped characterize skin lesions of patients with LET and supported the clinical findings that LET represents a distinct subset of CLE with a similar immunopathomechanism rather than a different disease. Alexiades-Armenakas et al. [17] performed a prospective study with 15 LET patients which were followed for a mean of seven years. Immunohistochemical findings showed that the majority of the cells in the inflammatory infiltrate reacted positively with an antibody to

Treatment

Due to the high photosensitivity, LET patients are recommended to avoid sun exposure in mid-day during summer and travel to sunny regions. Special consideration should be given to consistent UV protection through photoresistant clothing and application of light-shielding substances with highly potent chemical or physical UVA- and UVB-protective filters [47]. These substances should be applied in sufficient amount (ca. 2 mg/cm2) and with a high protection factor (SPF 50+) at least 15–30 min

Course of the disease

The clinical features observed during exacerbations of the disease activity in LET occurred in most cases in summer due to sun exposure, and some patients reported that even after sitting behind glass windows, their skin disease had worsened. This was confirmed in the study by Vieira et al. [16]; the majority of the 26 LET patients had mild recurrences, usually during spring or summer, related to the first sun exposure. More than 50% of the patients remained aysmptomatic only with adequate sun

Separate entity in the classification of CLE

In 1977, James Gilliam [53] initially proposed a nomenclature for the cutaneous manifestations of LE and soon thereafter, several refinements of this nomenclature were presented in collaboration with his colleague Richard Sontheimer [54], [55]. This clinical-histopathologic classification system divided all skin lesions that have some form of relationship to LE into those that are histologically specific for LE (LE-specific skin disease) and those that do not share this pattern of

Jessner's lymphocytic infiltration of the skin

LET bears striking similarities to “Jessner's lymphocytic infiltration of the skin” (JLIS) and its variant the “palpable migratory arciform erythema”, which are the most important differential diagnosis of LET. JLIS is a relatively uncommon relapsing disorder with asymptomatic or pruritic, papulonodular, nonscarring lesions without epidermal involvement that most often is located on the face (especially, on the zygomatic arch area) or upper back that has not always been considered a specific

Take-home messages

  • •

    LE tumidus (LET) has been characterized in the past by several groups demonstrating characteristic features different from other subtypes of cutaneous LE (CLE); in particular, phototesting has been crucial in confirming LET as a separate entity of the disease spectrum.

  • •

    Clinically, LET is characterized by succulent, urticaria-like, single or multiple plaques with a bright reddish or violaceous, smooth surface on sun-exposed areas. This subtype does never lead to long-term sequelae, such as

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    ☆

    The research was supported by a Heisenberg Scholarship from the German Research Foundation (DFG) to A. Kuhn (KU 1559/1-2).

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