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Lupus Erythematous Tumidus - Submitted on: Saturday September, 01, 2001
Contributed by:Artur Zembowicz, MD, Stephen Wang, MD
Lupus erythematous tumidus (LET)is a clinical entity believed to represent a rare non-scaring subset of chronic cutaneous lupus erythematosus. This disease was first described by Gougerot and Bournier in 1930, but was not again mentioned in literature until 1965.

Definition:
LET is a chronic erythematous annular dermatitis involving sun exposed skin, and is associated with perivascular and periadnexal lymphocytic infiltrate with dermal edema and mucin deposition. Epidermal and interface changes associated with discoid and subcutaneous lupus erythematosus is absent.

Clinical Features:
In the largest series published in the English literature, Kuhn et al reported a slight male predominance (55%), and the mean age of onset was 36.4 years. Clinically, LET presents as single or multiple erythematous, non-scarring, succulent, and urticarial-like plaques. Most of the lesions have no superficial changes. However, occasional scales can be observed. The lesions are localized predominantly in the upper extremity, face, upper back, and V area of the neck, indicating a photo-induced process. These lesions often disappear spontaneously, but can recur in the same area. In most cases, LET lesions are not associated with discoid lupus lesions. Patients with LET usually have normal serology, i.e., normal ANA, ds-DNA antibodies, anti-Ro, anti-La, anti-Scl-70, anti-Sm, and there is no correlation with systemic lupus erythematosus.

Histology:
Figures
(Click on an image for a larger view)
Figure 1
Figure 2
Figure 3
The classic histologic features of LET include perivascular and periadnexal lymphocytic infiltrate involving the superficial and deep dermis (Figure 1,2). Subepithelial edema and interstitial mucin deposition are present (Figure 3). Unlike the discoid lupus erythema and subacute cutaneous lupus erythema, LET lesions do not have follicular plugging, epidermal atrophy, vacuolar degeneration, or thickening of the basement membrane zone. Direct immunofluorescence staining yields negative results in LET lesions.


Treatment:
Many LET lesions spontaneously resolve with no residual scaring. Complete resolution of LET lesions can be achieved with application of topical steroids plus sunscreens with high sun-protection factor. For patients who do not respond to topical steroids, antimalarials, such as cholorquine phosphate, can be very effective.

Bibliography:
  • Kuhn A, Richter-Hintz D, Oslislo C, et al. Lupus Erythematous Tumidus. Arch Dermatol. 2000; 136:1033-1041.
  • Dekle CL, Mannes KD, Davis LS, et al. Lupus Tumidus. J Am Acad Dermatol. 1999;41:250-253.
  • Gougerot MH, Bournier. Lupus Erythematous Tumidus. Bull Soc Fr Derm Syph. 1930:1291-2.
     

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