To identify the underlying subtypes of hidradenitis suppurativa (HS), we performed latent class analysis on prospective clinical data of 618 consecutive patients seen between 2002 and 2010. The ...median patient age was 31 years (Q1=26; Q3=38), median age at HS onset was 20 years (16–25), and median Sartorius score was 18 (11–19); 34.4% of patients were of Hurley stage II or III. A three-class model showed the best fit. Latent class 1 (LC1) patients (48%) had a high probability of breast and armpit lesions (0.74) and hypertrophic scars (0.41). LC2 patients (26%) had a high probability not only of breast and armpit lesions (0.96) but also of lesions in the ears, chest, back, or legs (0.55); follicular lesions (pilonidal sinus: 0.48; comedones: 0.74); severe acne (0.47); and a family history of HS (0.44). Compared with LC1 patients, LC2 patients were more often male (odds ratio, 4.6; 95% confidence interval, 3–7; P<0.001) and current smokers (2.2; 1.3–3.9; P=0.005), and had greater disease severity (odds ratio, 1.6; 1.3–1.9; P<0.001). LC3 was characterized by gluteal involvement (0.54), papules, and folliculitis (0.71). LC3 patients were less often obese (0.6; 0.3–0.95; P=0.03) and had less severe disease (0.9; 0.7–1.1; P<0.001). These three phenotypes (“axillary–mammary”, “follicular”, and “gluteal”) may help stratify patients for clinical trials.
Background Conflicting opinions have been reported regarding the epidemiology of hidradenitis suppurativa. Objective We sought to evaluate the prevalence of hidradenitis suppurativa and to identify ...associated factors. Methodology Prevalence was evaluated using a representative sample of the French population (n = 10,000). Associated risk factors were assessed using two case-control studies, one population-based with 67 self-reported patients and 200 control subjects, and the other clinic-based with 302 medically assessed patients and 906 control subjects. Results The prevalence was 1% of the French population. Multivariate analyses showed a strong association with current smoking in self-reported (odds ratio = 4.16, 95% confidence interval 2.99-8.69) and in medically assessed (odds ratio = 12.55 8.58-18.38) populations. Association with body mass index was significant in medically assessed patients (odds ratio = 1.12 1.08-1.15) for each increase of 1 U of BMI. Limitations A causal relationship could not be established with such a cross-sectional study. Conclusion Hidradenitis suppurativa is a common disease, frequently associated with smoking and being overweight.
Sixty years after its original description by Sir Alan Lyell, epidermal necrolysis (from Stevens-Johnson syndrome to toxic epidermal necrolysis) seems finally amenable to a specific treatment in ...addition to essential symptomatic measures in specialized settings. A recently published systematic review and an article by Gonzales-Herrada et al. strongly suggest that cyclosporine is effective in reducing the risk of death.
Objective We sought to determine quality of life impairment in hidradenitis suppurativa. Methods Questionnaires were administered to 61 patients. Results Quality of life impact in hidradenitis was ...much greater than that of several other dermatologic conditions. Limitation This hospital-based population may not be representative. Conclusion Hidradenitis is one of the most distressing conditions observed in dermatology.
Background Factors associated with the severity of hidradenitis suppurativa (HS) are not known. Objective We sought to identify factors associated with the severity of HS. Methodology The severity of ...disease in a series of 302 consecutive patients with HS was assessed using the Sartorius score. Results Atypical locations were more common in men than in women (47.1% vs 14.8%; P < .001). Men also had more severe disease (median Sartorius score: 20.5 vs 16.5; P = .02). Increased body mass index ( P < .001), atypical locations ( P = .002), a personal history of severe acne ( P = .04), and absence of a family history of HS ( P = .06) were associated with an increased Sartorius score. The Sartorius score was highly correlated with the intensity and duration of pain and suppuration (all P values < .001). Limitations The referral center base of the study may have biased recruitment. Conclusion Our data showed a significant association between the severity of HS and several clinical and behavioral factors. Prospective studies are needed to confirm the prognostic role of these factors.
Three diagnostic criteria must be met for hidradenitis suppurativa: typical lesions, occurrence in one or more of the predilection areas, and that it is chronic and/or recurrent. Several outcome ...measures are used, including patient-reported pain and itch scales, Dermatology Life Quality Index, and Skindex. Hidradenitis suppurativa is associated with significant comorbidities that must be addressed in the evaluation of the patients.
Background The prognosis of toxic epidermal necrolysis (TEN), Stevens-Johnson syndrome (SJS), and SJS/TEN overlap syndrome has been assessed using a disease-specific severity score (SCORTEN) based on ...clinical and laboratory data. Histologic data may improve outcome prediction. Objective We sought to evaluate whether dermal mononuclear infiltration and epidermal necrosis predict survival of patients with TEN, SJS, or SJS/TEN. Methods We conducted a retrospective review of clinical records and skin biopsy specimens read without knowledge of clinical data. Results We identified 108 patients (SJS, n = 42; SJS/TEN, n = 36; TEN, n = 30). Overall mortality was 21.3%. Dermal infiltration and epidermal necrosis were not associated with time from disease onset to biopsy. Extensive dermal infiltrates were seen in 19 (18.5%) patients and full-thickness epidermal necrosis in 56 (52%) patients. Dermal infiltrate severity was not associated with day-1 (D1) SCORTEN or hospital death. Epidermal necrosis severity showed trends toward associations with D1 SCORTEN ( P = .11) and hospital death ( P = .06). In univariate analyses, full-thickness epidermal necrosis was significantly associated with hospital death (32.1% vs 11.4%, P = .017) and worse D1 SCORTEN values (1.98 ± 1.29 vs 1.55 ± 1.21; P = .04). In the bivariate analysis, however, D1 SCORTEN remained significantly associated with hospital death (odds ratio = 3.07, 95% confidence interval 1.83-5.16) but the association with full-thickness epidermal necrosis was no longer significant (odds ratio = 2.02, 95% confidence interval 0.65-7.12). Limitations Retrospective study design and indirect assessment of progression are limitations. Conclusion Full-thickness epidermal necrosis was associated with mortality but did not independently predict hospital death after adjustment based on the SCORTEN value. Dermal infiltrate severity was not associated with hospital death.
The SCORTEN, calculated within 24hours of admission, is a severity-of-illness score validated for toxic epidermal necrolysis and Stevens–Johnson syndrome. Our purpose was to assess the performance of ...successive SCORTEN during the first 5 days of hospitalization and to determine the influence of admission delay. Charts of 144 patients aged 46.8 years (±19.7), admitted to our department (1993–2003) with Stevens–Johnson syndrome or toxic epidermal necrolysis, were reviewed. Successive SCORTEN were compared between deceased patients (n=28, 19.4%) and survivors (n=116). The performance of the score (calibration, discrimination) was assessed on days 1–5. All seven SCORTEN variables, on days 1–5, were associated with a higher mortality rate. The SCORTEN rose slightly during hospitalization, with a significant difference between days 1 and 4 (<0.05). Performance of the SCORTEN was good on each day, but slightly better on day 3. The areas under the receiver-operating characteristic curves were above 80%. The admission delay did not differ between deceased patients and survivors. Delay-adjusted SCORTEN was close to the crude SCORTEN. The SCORTEN performance during the first 5 days of hospitalization was excellent, and at its best on day 3. We recommend to compute again the SCORTEN on day 3. The admission delay did not influence prognosis or SCORTEN.
The mortality of toxic epidermal necrolysis is about 30%. Our purpose was to develop and validate a specific severity-of-illness score for cases of toxic epidermal necrolysis admitted to a ...specialized unit and to compare it with the Simplified Acute Physiology Score and a burn scoring system. A sample of 165 patients was used to develop the toxic epidermal necrolysis-specific severity-of-illness score and evaluate the other scores, a sample of 75 for validation. Model development used logistic regression equations that were translated into probability of hospital mortality; validation used measures of calibration and discrimination. We identified seven independent risk factors for death and constituted the toxic epidermal necrolysis-specific severity-of-illness score: age above 40 y, malignancy, tachycardia above 120 per min, initial percentage of epidermal detachment above 10%, serum urea above 10 mmol per liter, serum glucose above 14 mmol per liter, and bicarbonate below 20 mmol per liter. For each toxic epidermal necrolysis-specific severity-of-illness score point the odds ratio was 3.45 (confidence interval 2.26–5.25). Probability of death was: P(death) = elogit/1 + elogit with logit = -4.448 + 1.237 (toxic epidermal nec-rolysis-specific severity-of-illness score). Calibration demonstrated excellent agreement between expected (19.6%) and actual (20%) mortality; discrimination was also excellent with a receiver operating characteristic area of 82%. The Simplified Acute Physiology Score and the burn score were also associated with mortality. The discriminatory powers were poorer (receiver operating characteristic area: 72 and 75%) and calibration of the Simplified Acute Physiology Score indicated a poor agreement between expected (9.1%) and actual (26.7%) mortality. This study demonstrates that the risk of death of toxic epidermal necrolysis patients can be accurately predicted by the toxic epidermal necrolysis-specific severity-of-illness score. The Simplified Acute Physiology Score and burn score appear to be less adequate.
BACKGROUND Thalidomide use in cutaneous sarcoidosis is based on data from small case series or case reports. The objective of this study was to evaluate the efficacy and safety of thalidomide in ...severe cutaneous sarcoidosis. METHODS This study consisted of a randomized, double-bind, parallel, placebo-controlled, investigator-masked, multicenter trial lasting 3 months and an open-label study from month 3 to month 6. Adults with a clinical and histologic diagnosis of cutaneous sarcoidosis were included in nine hospital centers in France. Patients were randomized 1:1 to oral thalidomide (100 mg once daily) or to a matching oral placebo for 3 months. In the course of an open-label follow-up from month 3 to month 6, all patients received thalidomide, 100 mg to 200 mg daily. The proportions of patients with a partial or complete cutaneous response at month 3, based on at least a 50% improvement in three target lesions scored for area and infiltration, were compared across randomization groups. RESULTS The intent-to-treat population included 39 patients. None of them had a complete cutaneous response. Four out of 20 patients in the thalidomide group (20%) vs four out of 19 patients in the placebo group (21%) had a partial cutaneous response at month 3 (difference in proportion of −1% 95% CI, −26% to +24% for thalidomide vs placebo, P = 1.0). Eight patients with side effects were recorded in the thalidomide group vs three in the placebo group. We observed a large number of adverse event-related discontinuations in patients taking thalidomide in the first 3 months (four patients with thalidomide, zero with placebo) and in the 3 following months (five patients). CONCLUSIONS At a dose of 100 mg daily for 3 months, our results do not encourage thalidomide use in cutaneous sarcoidosis. TRIAL REGISTRY ClinicalTrials.gov ; No.: NCT0030552; URL: www.clinicaltrials.gov
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