Articulo de reumatologia

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Lupus

Lupus erythematosus tumidus: an underestimated entity
M Teixeira, M Ferreira, R Alves and M Selores Lupus 2006 15: 296 DOI: 10.1191/0961203306lu2296cr The online version of this article can be found at: http://lup.sagepub.com/content/15/5/296

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Lupus (2006) 15, 296–300www.lupus-journal.com

CASE REPORT

Lupus erythematosus tumidus: an underestimated entity
M Teixeira*, M Ferreira, R Alves and M Selores
Department of Dermatology, Hospital Geral de Santo António, Porto, Portugal

There have been few reports of lupus erythematosus tumidus (LET) in the literature. Most of textbooks of dermatology or dermatopathology mention this entity only briefly, if at all. The authorsdescribe an additional case of this underdiagnosed disorder that further supports its existence as a separate entity in the spectrum of the variants of chronic cutaneous lupus erythematosus. Although most cases are reported in the European countries, to our knowledge, this is the first case documented in Portugal. The clinical, photobiological and histological features as well as differential diagnosis,treatment and prognosis are also discussed. Lupus (2006) 15, 296–300 Key words: cutaneous lupus erythematosus; face; hydroxicloroquine; lupus erythematosus tumidus

Introduction
Lupus erythematosus (LE) is an autoimmune disorder with a wide spectrum of clinical expression extending from a localized cutaneous form to a life-threatening systemic one.1 According to Sontheimer, cutaneous LE (CLE)can be classified as acute LE (ACLE), subacute LE (SCLE) and chronic CLE (CCLE). The most common subtype of CCLE is discoid LE (DLE), and there are additional rare varieties of CCLE, such as the tumid type, the hypertrophic type, the profundus type and chilblain lupus.2 Since the course and severity of the different subtypes of CLE is different in each patient, it is always necessary to evaluatesystemic involvement. The American Rheumatology Association criteria published in 1982 are currently in use.3 The term ‘Lupus erythematosus tumidus’ was first mentioned in 1909 by Hoffman in the Berlin Dermatological Society,4 and in 1930, five further patients were reported by Gourgerot and Bournier.5 LET is a distinct variety of CCLE that occurs with much less frequency than other forms of cutaneouslupus. Although recently, new cases of LET have been described indicating a probable higher prevalence that initially thought. Its hallmark clinical features are succulent, urticaria-like, single or multiple plaques with a bright reddish or violaceous, smooth surface on sun-exposed areas. The plaques often clear

spontaneously without leaving scars and then recur in the originaldistribution.6,7

Case report
A 62-year old white woman, rural worker, presented with a six-year history of asymptomatic bright-red, elevated plaques on her nose, front and malar regions (Figure 1a and b). These skin lesions would come and go always in the same location without scarring and were exacerbated by sun exposure. She also complained of sporadic arthralgia on both knees. Medical history wassignificant for dislipidemia, but she was on no medications. Personal and familiar medical history was otherwise irrelevant. A lesional skin biopsy specimen revealed a perivascular and periadnexal lymphocytic infiltrate, papilar dermal edema (Figure 2). Intersticial mucin deposition in the reticular dermis was observed using Alcian blue staining (Figure 3). Direct immunofluorescence using complement...
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