Lupus erythematosus acute-cutaneous L93.1

Non-scarring, photosensitive, acute to subacute, mostly recurrent manifestation of cutaneous lupus erythematosus. The relapses are accompanied by symptoms of varying severity. The facial "butterfly erythema" is an example of this form of non-scarring cutaneous lupus erythematosus. Erythema-multiforme-like symptoms are rarer.

The association with the occurrence of anti-ds DNA antibodies is described.

Initial manifestation 20-50 years. In larger groups of patients with cutaneous lupus erythematosus (CL) about 1/3 ACLE patients are detected. Transitional forms can be observed as well as the synchronous occurrence of several subtypes in one individual.

Centrofacial parts of the face with emphasis on the cheek areas; also generalized distribution with involvement of the face, trunk and extremities.

Frequently after exposure to the sun, but also spontaneously occurring butterfly-shaped erythema in the area of both cheeks, persisting for a few days or remaining for weeks, soft to bright red, with discreet to absent scaling. Rare infestation of the trunk and the upper extremity. Healing without scarring, possibly with pigmentary shifts. Not uncommon are exanthematic courses of disease with a symmetrical pattern of infestation. Anular patterns are observed in these phases of relapse in detail morphologically. These are mainly observed on the trunk and the upper extremity, less so on the lower extremity.

Rarely (in a larger collective of SJS/TEN patients this was 0.6% - Ziemer M et al. 2012) do patients develop severe, possibly life-threatening courses reminiscent of Stevens-Johnson syndrome (old name = Rowell`s syndrome) or toxic epidermal necrolysis (TEN-like ACLE).

Non-specific; the occurrence of anti-Ro/SSA and anti-La/SSB antibodies has been described; furthermore, also inconstant anti-dsDNA antibodies possibly combined with lupus nephritis.

Depending on the severity of the symptoms, glucocorticoids locally or systemically in medium doses(prednisolone derivatives).

There is experience in larger collectives with rituximab.

1. Baker MG et al (2014) Systemic lupus erythematosus presenting as Stevens-Johnson syndrome/toxic epidermal necrolysis. J Clin Rheumatol 20:167-171
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. Eastham AB et al (2013) Cutaneous lupus erythematosus. JAMA Dermatol 150:344
4. Grönhagen CM et al (2014) Cutaneous lupus erythematosus: An update. Indian Dermatol Online J 5:7-13
5. Kuhn Aet al. (2014) The classification and diagnosis of cutaneous lupus erythematosus. J Autoimmune PubMed PMID: 24486120
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7. Roustan G et al (2000) Lupus erythematosus with an erythema exsudativum multiforme-like eruption. Eur J Dermatol 10: 459-462
8. Vital EM et al (2015) Responses to rituximab suggest B cell-independent inflammation in cutaneous systemic lupus erythematosus. Am J Hematol doi: 10.1002/ajh.23993
9. Ziemer M et al (2012) Stevens-Johnson syndrome and toxic epidermal necrolysis in patients with lupus erythematosus: a descriptive study of 17 cases from a national registry and review of the literature. Br JDermatol 166: 575-600