Cutaneous lupus erythematosus (overview) L93.-

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 03.01.2021

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Synonym(s)

CLE; Cutaneous lupus erythematosus; Hautlupus; Lupus erythematosus integumentalis; Lupus erythematosus of the skin

History
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In 1845, F. v. Hebra described the clinical picture of cutaneous lupus erythematosus precisely for the first time under the name of "Seborrhoea congestiva" according to information from M. Kaposi (see illustration). as "Lupus erythematosus". In earlier publications of Laurent Théodore Biett under the designation "datre rongeante qui détruit en surface" was also referred to this clinical picture, to which M. Cazenave and H.E. Schedel refer in their "Abrégé pratique des mal. des la peau" from the year 1828. Thus, Laurent Théodore Biett (1781-1840; active at the Hospital St. Louis, there successor of Jean Louis Marc Alibert) probably deserves the honor of the first description, whereby (as M. Kaposi writes) "the clinical picture and the naming of the disease in a characteristic way was first established by Hebra, extended and named by Cazenave, according to later experiences".

Definition
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Cutaneous lupus erythematosus (CL) is an acute, sub-acute or chronic inflammatory autoimmune disease dominated by skin symptoms with a broad spectrum of clinical manifestations and a variable course. Depending on the acuteity and depth of the infiltrate cells, different subtypes can be distinguished. Important: Transitional forms are observed as well as the synchronous occurrence of several subtypes in one individual.

Classification
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A generally valid classification of cutaneous lupus erythematosus (CLE) goes back to Gilliam and Sontheimer (1981), who distinguished between specific and non-specific lesions of cutaneous LE. In the course of the disease, a tripartite division can be made according to acuities. Accordingly, a distinction is made between:

  • Acute
  • Subacute
  • Chronic

The following clearly defined clinical syndromes are classified in these categories. The numbers in brackets refer to their frequencies within a collective of CL patients (Biazar C et al. 2013):

Occurrence/Epidemiology
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Worldwide occurrence; the incidence of cutaneous lupus erythematosus is about 10 times higher than that of systemic lupus erythematosus (SLE).

Etiopathogenesis
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It is assumed that in cutaneous LE (CLE) epidermal keratinocytes are the target cell of the "immunological injury". It is postulated that an autoimmunological reaction is triggered by increased apoptosis induction (keratinocytes of LE patients produce increased IL-18 receptors on their surface after stimulation with TNF-α or IFN-γ; they become more apoptotic after IL-18 exposure. At the same time, the production of IL-12 is reduced; IL-12 protects keratinocytes from UV-induced apoptosis).

Manifestation
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Mainly occurring in middle-aged adults (25 - 60 years, mean: 43+/- 16 years), more frequent in women (w:m=3-6:1)

Clinical features
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See below the individual clinical forms.

Laboratory
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Rarely antinuclear factors or cardiolipin antibodies, leukopenia ( autoantibodies).

Histology
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See below the individual clinical forms.

Complication(s)
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Mutilations, irreversible alopecia, poorly healing ulcerations with development of spinocellular carcinomas, among others, can occur.

Therapy
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According to the lupus erythematodes chronicus discoides and the individual clinical variants, see there.

Progression/forecast
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Chronic course over years, defect healing. In about 5% transition to systemic lupus erythematosus.

Tables
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Clinical forms of integumentary lupus erythematosus:

Diet/life habits
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In all forms of lupus erythematosus, increased sensitivity to both UVB and UVA (less frequently to visible light) is found. Strict light protection is therefore necessary. After UV provocation, the reactions mostly occur after one week or later.

Note(s)
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For clinical studies, some authors propose the "Cutaneous Lupus Erythematodes Disease Area and Severity Index" (CLASI), which considers localization, erythema, scaling and infiltration, dyspigmentation and scarring.

Literature
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  1. Albrecht J et al (2005) The CLASI (cutaneous lupus erythematosus disease area and severity index): an outcome instrument for cutaneous lupus erythemtosus. J Invest Dermatol 125: 889-894
  2. Biazar C et al (2013) EUSCLE co-authors. Cutaneous lupus erythematosus: first multicenter database analysis of 1002 patients from the European Society of Cutaneous Lupus Erythematosus (EUSCLE). Autoimmune Rev 12:444-454
  3. Bohmeyer J et al (2002) Thalidomide therapy of cutaneous lupus erythematosus. Dermatologist 53: 744-748
  4. Fabbri P et al (2003) Cutaneous lupus erythematosus: diagnosis and management. Am J Clin Dermatol 4: 449-465.
  5. Gilliam JN, Sontheimer RD (1981) Distinctive cutaneous subsets in the spectrum of lupus erythematosus. J Am Acad Dermatol 4: 471-475
  6. Kaposi M(1976) Lupus erythematosus. In Virchow R (ed) Handburch der speciellen Pathologie und Therapie. Verlag F. Enke, Stuttgart pp.298, 299-323.
  7. Child P et al (1992) Cutaneous lupus erythematosus and cardiolipin antibodies. Dermatologist 43: 126-129
  8. Kuhn A et al (2000) Rare cutaneous manifestations of lupus erythematosus. A clinical overview. Dermatologist 51: 818-825
  9. Sander CJG (2003) Phtosensitivity in patients wiith lupus erythematosus: a clinical and photobiological study in 100 patients using a prolonged phototest protocol. Br J Dermatol 149: 131-137
  10. Wang D et al (2008) Evidence for a pathogenetic role of interleukin-18 in cutaneous lupus erythematosus. Arthritis Rheum 58: 3205-3215

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 03.01.2021