Cutaneous lupus erythematosus (overview) L93.-

In 1845, according to M. Kaposi (see illustration), F. v. Hebra precisely described the clinical picture of cutaneous lupus erythematosus for the first time under the name "seborrhoea congestiva", which was later (1851) named "lupus erythematosus" by M. Cazenave in the "Annales des maladies de la peau". In earlier publications by Laurent Théodore Biett, this clinical picture was also referred to as "datre rongeante qui détruit en surface", to which M. Cazenave and H.E. Schedel refer in their "Abrégé pratique des mal. des la peau" from 1828. Thus, Laurent Théodore Biett (1781-1840; worked at the St. Louis Hospital, where he succeeded Jean Louis Marc Alibert) probably deserves the honor of the first description, whereby (as Moriz Kaposi writes) "the clinical picture and the name of the disease was first established in a characteristic manner by Hebra, and was expanded and named by A . Cazenave in accordance with later experience".

Cutaneous lupus erythematosus (CL) is an acute, subacute, or chronic inflammatory autoimmune disease dominated by skin symptoms, with a wide spectrum of clinical manifestations and a variable course. Different subtypes can be distinguished depending on the acuity and depth of the infiltrate cells. Important: Transitional forms can be observed as well as the synchronous occurrence of several subtypes in one individual.

A generally valid classification of cutaneous lupus erythematosus (CLE) goes back to Gilliam and Sontheimer (1981), who differentiate between specific and non-specific lesions of cutaneous LE. The course of the disease can be divided into three categories according to acuteity. 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):

• Acute cutaneous lupus erythematosus (ACLE - 30%):
• Subacute cutaneous lupus erythematosus (SCLE - 24%)
• Chronic cutaneous lupus erythematosus (CDLE):
  • Lupus erythematosus chronicus discoides - localized/disseminated - (CDLE - 40%)
  • Lupus erythematosus profundus (lupus erythematosus panniculitis).
• Intermittent cutaneous lupus erythematosus
  • Lupus erythematosus tumidus
• Rare special forms:
  • Chilblain's lupus
  • Rowell's syndrome (to be defined as erythema exudativum-multiforme-like ACLE - see below) Lupus erythematosus acute-cutaneous)
  • Lupus erythematosus verrucosus
  • Neonatal lupus erythematosus

Worldwide incidence; the incidence of cutaneous lupus erythematosus is approximately 10x more common than systemic lupus erythematosus (SLE). The incidence of systemic lupus erythematosus is reported to be 5-10/100,000/year.

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

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

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

Clinical forms of integumentary lupus erythematosus:

• Lupus erythematosus discoides
• Lupus erythematosus chronicus integumentalis cum exacerbatione acuta
• Lupus erythematosus chronicus superficiales disseminatus
• Lupus erythematosus profundus
• Lupus erythematosus hypertrophicus et profundus
• Lupus erythematosus verrucosus
• Lupus erythematosus tumidus
• Chilblain's lupus

For clinical studies, some authors suggest the determination of the "Cutaneous Lupus Erythematosus Disease Area and Severity Index" (CLASI), which takes into account localization, erythema, scaling and infiltration, dyspigmentation and scarring.

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