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

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.

Characteristics of patients with late onset systemic lupus erythematosus in Turkey.

Systemic lupus erythematosus (SLE) is a multi-system autoimmune disorder mainly affecting young women. In this study, we aimed at investigating the clinical, laboratory and management characteristics of our SLE patients with an age of onset > or =50. Twenty patients with late onset SLE (> or =50 years) were identified from the records, on the basis of their first SLE-related symptoms (Group I). A hundred consecutive SLE patients with initial symptoms before the age of 50 were also selected as controls (Group II). Clinical, laboratory and management characteristics of the patients were recorded according to pre-defined protocol and compared by chi(2), Student's t-test and Fisher's exact test. Comparison of the demographic findings between the Group I (F/M: 18/2) and the Group II (F/M: 90/10) were as follows: the mean age of disease onset was 53.9 +/- 4.5 years vs. 26.3 +/- 9.2 years, mean time of follow-up was 44.2 +/- 40.5 months vs. 50.1 +/- 47.4 months, mean damage index was 0.6 +/- 0.6 vs. 0.58 +/- 1.4. There was no significant difference between the two groups with regard to clinical, laboratory parameters, damage index and immunosuppressive treatment characteristics. SLE-related manifestations were similar in two groups except fever (10% in the Group I vs. 41% in the Group II; p = 0.01). The only two patients with pulmonary fibrosis were found in the Group I (p = 0.027). The clinical and laboratory characteristics and the disease outcome in SLE patients with an age of onset > or =50 years did not show significant differences from the control SLE patients with a younger age of onset.

Characteristics of patients with adult-onset familial Mediterranean fever in Turkey: analysis of 401 cases.

It has been generally accepted that the clinical onset of familial Mediterranean fever (FMF) begins before 20 years of age in most patients. In this study, we aimed to investigate the demographic and clinical characteristics of our FMF patients with an age of onset > or =20. Records of 401 patients (female/male: 204/197) that followed up between 1990 and 1999 were reviewed according to a pre-defined protocol. All patients fulfilled the diagnostic criteria of Livneh et al. The demographic and clinical features of adult-onset FMF patients were compared to those of patients with a disease onset before 20 years of age. There were 57 patients (14%) who experienced symptoms of FMF at > or =20 years of age; 34 of them (8.5%) reported their first attack between 20 and 29 years of age; 18 of them (4.5%) between 30 and 39 years of age and five patients (1.25%) had their first attack after 40 years of age. Arthritis (42 vs. 65%, p = 0.001) and erysipelas-like erythema (7 vs. 17%, p = 0.047) were significantly less frequent in patients with adult-onset FMF compared to patients with disease onset before 20 years of age. Arthritis and erysipelas-like erythema were less frequent in adult-onset patients compared to those with an earlier disease onset. Adult-onset FMF may be a form of disease with distinct clinical, demographic and molecular characteristics. Prospective clinical studies and investigation of genotypic features are needed to identify the characteristics of this phenotypic variant.

Tuberculosis in Turkish patients with systemic lupus erythematosus: increased frequency of extrapulmonary localization.

The objective was to investigate the frequency and characteristics of tuberculosis (TB) in patients with systemic lupus erythematosus (SLE). We reviewed the charts of 556 patients with SLE who were followed up between 1978 and 2001 in our lupus clinic. Patients who developed TB after the diagnosis of SLE were identified (SLE/TB+). Ninety-six consecutive patients with SLE who did not develop TB during the follow-up were evaluated as a control group (SLE/TB-). Clinical, laboratory and management characteristics of these two groups of patients were recorded according to a predefined protocol, and compared. Of the 556 patients evaluated, 20 patients (3.6%) with TB were identified. Nine of the 20 patients (45%) had extrapulmonary TB (vertebral involvement in three patients, meningeal in two, and joint and soft tissue in four). Arthritis and renal involvement were significantly high in the SLE/TB+ group (P = 0.045, P = 0.009, respectively). The mean daily dose of prednisolone before the diagnosis of TB and the cumulative dose of prednisolone were significantly higher in the SLE/TB+ group compared to the SLE/TB- group (27 +/- 22 g versus 16 +/- 16 g, 24 +/- 45 mg versus 11 +/- 8.5 mg, respectively). In conclusion, we found an increased frequency of TB infection and a high prevalence of extrapulmonary TB in a large cohort of SLE patients. The mean daily dose of prednisolone before the diagnosis of TB and the cumulative dose of prednisolone, which possibly related to disease severity, were important determinants for the increased risk of TB in these patients with SLE.

Duplex and color Doppler sonographic findings in active sacroiliitis.

OBJECTIVE: The aim of this study was to describe the duplex and color Doppler sonographic findings in active sacroiliitis. SUBJECTS AND METHODS: Forty-one joints in 21 patients with active sacroiliitis, 20 sacroiliac joints in 10 patients with osteoarthritis, and 30 sacroiliac joints of 15 asymptomatic volunteers were investigated on duplex and color Doppler sonography. We investigated whether a vessel was present around the posterior portions of sacroiliac joints with color Doppler sonography. When an artery was detected, the resistive index (RI) was measured using duplex Doppler sonography in all groups and also after treatment in the patients with active sacroiliitis. RESULTS: Vascularization around the posterior portions of sacroiliac joints was seen in 41 joints of the 21 patients with active sacroiliitis, nine joints of six patients with osteoarthritis, and 13 joints of eight volunteers. The mean RI values were 0.62 +/- 0.13, 0.91 +/- 0.09, and 0.97 +/- 0.03, respectively. In the patients with active sacroiliitis, the mean RI value was 0.91 +/- 0.07 after therapy. The RI values for the patients with active sacroiliitis were significantly different from those of the patients with osteoarthritis (p < .001) and of the volunteers (p < .001). In addition, the RI values were significantly different before and after treatment in the patients with active sacroiliitis (p < .001). CONCLUSION: Vascularization around the posterior portions of sacroiliac joints increased and RI values decreased in patients with active sacroiliitis. Color and duplex Doppler sonography were able to reveal these changes and can be used in the diagnosis of active sacroiliitis and follow-up after treatment. Thus, RI values may be a quantitative indicator for clinical symptoms in patients with active sacroiliitis.