A case of toxic epidermal necrolysis-like skin lesions with systemic lupus erythematosus and review of the literature

• Published in: Lupus 2013-07, Vol.22 (8), p.839-846
• Main Authors: Cetin, G Yildirim, Sayar, H, Ozkan, F, Kurtulus, S, Kesici, F, Sayarlıoglu, M
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Antibodies, Antinuclear - blood; Biopsy; Biopsy - methods; Case reports; Child; Disease Progression; Female; Hospitals; Humans; Internal medicine; Literature reviews; Lupus; Lupus Erythematosus, Cutaneous - diagnosis; Lupus Erythematosus, Cutaneous - pathology; Lupus Erythematosus, Systemic - complications; Lupus Erythematosus, Systemic - diagnosis; Lupus Erythematosus, Systemic - pathology; Male; Middle Aged; Patients; Pleural Effusion - etiology; Rheumatology; Skin; Stevens-Johnson Syndrome - diagnosis; Stevens-Johnson Syndrome - etiology; Stevens-Johnson Syndrome - pathology; Tomography; Young Adult
• ISSN: 0961-2033; 1477-0962
• DOI: 10.1177/0961203313492242

Objective Subepidermal bullous lesions and toxic epidermal necrolysis-like (TEN-like) lesions can occur in patients with systemic lupus erythematosus (SLE). In this report, we describe a case of a patient previously diagnosed with SLE who experienced TEN-like skin lesions with unusual subacute progression in the context of the current literature. Methods We present a recent case of TEN-like lupus erythematosus and review of studies published in English identifying SLE cases mimicking TEN, accessed via PubMed and Google Scholar databases. The keywords used in the search were: TEN, SLE, TEN-like SLE, and TEN-like lesions. The search covered all articles from January 1980 to November 2011. Results A 52-year-old male presented with fatigue, weakness, and weight loss (23 kg in two months). Skin redness started across nose and cheeks six months before admission. Bilateral pleural effusions were observed in a thorax tomography taken in the referral hospital two months prior to admission. Because of articular involvement, antinuclear antibody (ANA), and anti-dsDNA positivity, the patient was diagnosed with SLE. We initiated a punch skin biopsy, and the findings were consistent with Stevens-Johnson syndrome. There was marked basal layer necrosis in the epidermis, and there was predominantly lymphohistiocytic infiltrate in the dermis. A total of 22 cases, including our case, with TEN-like lupus erythematosus were reported in the literature. In addition, cutaneous lupus had positive ANAs in 18 of 22 patients (81.8%). The patients were aged 12 to 76 years; 21 cases were women and only one patient was male. Discussion Skin involvement, including the rare variant of TEN-like acute cutaneous SLE, is very common among SLE patients. The acute syndrome of pan-epidermolysis or apoptotic pan-epidermolysis may become a useful designation when considering a clinical diagnosis of drug-induced TEN or SLE. Further studies are required to verify our findings.