Lupus tumidus
ICD-10 L93 · ICD-11 EB51.Y.1

Severe or Disseminated Cutaneous Lupus Erythematosus Skin Lesions Not Responding After First-Line Treatment

This protocol addresses patients with lupus tumidus presenting with severe or disseminated cutaneous lupus erythematosus (CLE) skin lesions whose condition has not adequately improved following a complete first-line treatment course.

Clinical scenario
Severe or disseminated cutaneous lupus erythematosus skin lesions — a presentation for which antimalarial drugs are recommended as first-line treatments in all CLE patients, particularly those at risk of scarring, combined with systemic glucocorticoids for a limited period.
First-line treatment — failure condition

The prior regimen included topical glucocorticoids (class 2–4), or topical tacrolimus, or topical pimecrolimus, combined with hydroxychloroquine or chloroquine, and a tapering course of prednisolone.

Escalation to this protocol is indicated when no adequate improvement of cutaneous lupus erythematosus skin lesions is observed after 16 weeks of that regimen.

Next-step approach (partial overview)
This next-line protocol introduces the addition of a further systemic agent — mepacrine — used in combination with an antimalarial drug. The complete regimen, sequencing, and full prescribing details are in the structured protocol below.

Treatment goals: Improvement of skin lesions within 3–4 weeks; maximum effects expected after 6–8 weeks.

References

DOI: 10.1111/ddg.14491

Antimalarial drugs are recommended as first-line treatments, also for long-term therapy, in all CLE patients with severe and disseminated skin lesions; in particular for patients with a risk of scarring.

For severe or disseminated CLE lesions, systemic glucocorticoids are recommended as first-line treatment in addition to antimalarial drugs, for a limited period of time.

In treatment refractory cases, or in cases of intolerance or retinopathy, systemic treatment with mepacrine is suggested either instead of or in combination with HCQ or CQ.

Mepacrine is usually combined with CQ/HCQ since it acts synergistically with these drugs and does not increase the risk of retinopathy.

Skin lesions will improve within three to four weeks; maximum effects are seen after six to eight weeks.

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