Summary
Lupus tumidus is a rare immunological disorder whose pathogenesis is not fully understood. Although on the one hand there are some differences in (1) the clinical morphology of lesions, (2) the histopathology picture, as well as in (3) serological profile of lupus tumidus patients at the current moment, the disease is regarded as a subform of chronic cutaneous lupus erythematosus. Differential diagnosis requires the exclusion of many diseases such as Jessner Kanoff lymphocytic infiltration, polymorphous light eruption, and reticular erythematous mucinosis. Differentiation between them is not always easy. The standard treatment regimen of patients with lupus tumidus is based on local and systemic application of corticosteroids, azathioprin, dapsone, mepacrine, chloroquine, and hydroxychloroquine. Modern treatment options include the use of pulsed dye laser, tacrolimus 0.1 % ointment and photophoresis. We present a 42-year-old patient with unilateral atypical form of lupus tumidus who was successfully treated with systematically administration of hydroxychloroquine in combination with sunscreen SPF 50. After a 2-month course of treatment with hydroxychloroquine dosage of 200 mg per day and a break of 3 months between courses, we observed a complete remission.
Zusammenfassung
Lupus tumidus ist eine seltene Immunkrankheit, deren Pathogenese nicht vollständig aufgeklärt ist. Trotz einiger Unterschiede 1) im klinischen Erscheinungsbild, 2) in der Histopathologie und 3) im serologischen Profil wird die Erkrankung als Unterform des chronisch-kutanen Lupus erythematodes eingeordnet. In der Differentialdiagnose sind zahlreiche Erkrankungen auszuschließen wie die lymphozytäre Infiltration Jessner-Kanoff, die polymorphe Lichtdermatose und die retikuläre erythematöse Muzinose (REM-Syndrom). Die Unterscheidung zwischen diesen ist nicht immer leicht. Die Standardtherapie für Patienten mit Lupus tumidus basiert auf der topischen und systemischen Gabe von Kortikosteroiden, Azathioprin, Dapson, Mepacrin, Chloroquin und Hydroxychloroquin. Moderne Behandlungsoptionen sind auch gepulste Farbstoff-Laser, Photopherese und topisches Tacrolimus 0,1%. Wir berichten über einen 42-jährigen Patienten mit einer unilateralen atypischen Variante des Lupus tumidus, der erfolgreich mit Hydroxychloroquin 200 mg/d in Verbindung mit Lichtschutz (SPF 50) behandelt wurde. Nach 2-maliger Behandlung über 8 Wochen mit einer therapiefreien Pause von 12 Wochen erzielte der Patient eine Komplettremission.
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IB, AAC, IK, CT, UW, TL, and GT declare that there are no actual or potential conflicts of interest in relation to this article.
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Bakardzhiev, I., Chokoeva, A.A., Krasnaliev, I. et al. Unilateral unique Lupus tumidus: pathogenetic mystery and diagnostic problem. Wien Med Wochenschr 166, 250–253 (2016). https://doi.org/10.1007/s10354-015-0421-2
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DOI: https://doi.org/10.1007/s10354-015-0421-2