L. Vaillant

Acute generalized exanthematous pustulosis (AGEP) – A clinical reaction pattern

Background: A wide range of diseases or reactions can cause pustular eruptions of the skin. In this spectrum there seems to be a subgroup with characteristic clinical features and a typical course which is mostly caused by drugs for which the term acute generalized exanthematous pustulosis (AGEP) has been established.

Risk factors for erysipelas of the leg (cellulitis): case-control study

Abstract Objective: To assess risk factors for erysipelas of the leg (cellulitis). Design: Case-control study. Setting: 7 hospital centres in France. Subjects: 167 patients admitted to hospital for erysipelas of the leg and 294 controls. Results: In multivariate analysis, a disruption of the cutaneous barrier (leg ulcer, wound, fissurated toe-web intertrigo, pressure ulcer, or leg dermatosis) (odds ratio 23.8, 95% confidence interval 10.7 to 52.5), lymphoedema (71.2, 5.6 to 908), venous insufficiency (2.9, 1.0 to 8.7), leg oedema (2.5, 1.2 to 5.1) and being overweight (2.0, 1.1 to 3.7) were independently associated with erysipelas of the leg. No association was observed with diabetes, alcohol, or smoking. Population attributable risk for toe-web intertrigo was 61%. Conclusion: This first case-control study highlights the major role of local risk factors (mainly lymphoedema and site of entry) in erysipelas of the leg. From a public health perspective, detecting and treating toe-web intertrigo should be evaluated in the secondary prevention of erysipelas of the leg.

Neurological disorders in patients with bullous pemphigoid.

Background: Unexpected cases of bullous pemphigoid (BP) have been reported in adult patients with various neurological disorders suggesting a possible relationship between these diseases. Objectives: (1) To determine the prevalence and types of neurological disorders in patients with BP, (2) to assess patients’ functional impairment, and (3) to compare the clinical and biological findings as well as prognosis of BP patients presenting with or without neurological disorders. Methods: BP patients with neurological disorders were selected in a series of 341 consecutive BP patients treated in 20 French Dermatology Departments. Functional impairment was prospectively assessed using the Karnofsky score which is a measure of patients’ general condition. Results: At least one neurological disorder was present in 123 of the 341 BP patients (36%). They primarily consisted of dementia (n = 68; 20%; 95% CI: 16–25%), cerebral stroke (n = 52; 15%; 95% CI: 4–19%), and/or Parkinson’s disease or parkinsonism (n = 32; 9%; 95% CI: 7–13%). BP patients with neurological disease were older than patients without neurological disease (83.8 ± 7.5 years vs. 79.3 ± 10.3 years, p < 10–4). They also had a lower Karnofsky score (47 ± 19% vs. 74 ± 20%, p < 10–4). One-year overall survival rates of the two groups were 50.8% (95% CI: 41.8–59.7) and 78.7% (95% CI: 73.0–84.2), respectively (p < 10–4). In contrast, the number of bullae and main biological features at baseline were not different between the two groups of patients. Conclusion: This study demonstrated a high frequency of neurological disorders, particularly dementia, in BP patients. Most of these patients had a severe functional impairment and a poor prognosis.

Risk Factors for Relapse in Patients With Bullous Pemphigoid in Clinical Remission: A Multicenter, Prospective, Cohort Study

OBJECTIVE To identify prognostic factors for relapse in the first year after cessation of therapy in bullous pemphigoid (BP). DESIGN Prospective, multicenter, cohort study (January 1, 2000, through December 31, 2006). SETTING Fifteen French dermatology departments. Patients Patients with BP in remission under low doses of topical or systemic corticosteroids. Interventions Cessation of corticosteroid treatment (day 0) followed by a systematic clinical and immunologic follow-up. MAIN OUTCOME MEASURES The end point was clinical relapse within the first year after cessation of therapy. Associations of clinical, biological, and immunologic (including direct immunofluorescence, serum anti-basement membrane zone autoantibodies, and serum BP180 autoantibodies by enzyme-linked immunosorbent assay [ELISA] on day 0) variables with clinical relapse were assessed by means of univariate and multivariate analyses. RESULTS On day 0, 30 of 114 patients (26.3%) still had a positive result of direct immunofluorescence, 63 of 112 (56.3%) had circulating anti-basement membrane zone autoantibodies, and 34 of 57 (60%) had anti-BP180 antibodies by ELISA. At month 12, 22 patients were dead (n = 11) or lost to follow-up (n = 11), 51 were in remission, and 45 had had relapses (mean interval to relapse, 3.2 months). Factors predictive of relapse within 12 months after cessation of therapy were a positive result of direct immunofluorescence microscopy (P = .02), a greater age (P = .01), and high-titer ELISA scores (P = .02) on day 0. In multivariate analysis, the only factor independently predictive of relapse was a high-titer ELISA score on day 0 (odds ratio, 11.00; 95% confidence interval, 1.29-93.76). CONCLUSIONS High-titer anti-BP180 ELISA score and, to a lesser degree, a positive direct immunofluorescence finding are good indicators of further relapse of BP. At least 1 of these tests should be performed before therapy is discontinued.

Is ultrasound lymph node examination superior to clinical examination in melanoma follow-up? A monocentre cohort study of 373 patients.

Background  There is still lack of consensus regarding the most effective follow‐up for stage I and II melanoma patients although some consensus conferences have provided guidelines stating that clinical examination should be the standard.

Cutaneous alternariosis: role of corticosteroid-induced cutaneous fragility.

Alternaria is a very common and saprophytic fungus. Cutaneous infection is rare and about 71 cases have been described, mainly in Europe in immunocompromised hosts. We report a case of dermal alternariosis occurring in a woman treated with corticosteroids for dermatomyositis. The cutaneous lesion consisted of an erythematous and scaly plaque on the leg measuring 2 x 2 cm. Cutaneous biopsy showed hyphae and round inclusions stained with PAS and Gomori-Grocott within a polymorphous granuloma. Cultures of cutaneous biopsies grew Alternaria sp. HIV1 and HIV2 serology was negative. The patient was treated by local excision and corticosteroids were decreased. One-year follow-up showed no recurrence. Cutaneous alternariosis is an opportunistic infection. the disease has been described mainly in patients treated with systemic corticosteroids (39 cases out of the 71 reported cases) or local corticosteroids (3/71) and in patients suffering from Cushings syndrome (7/71) but rarely in HIV-infected patients (3/71). Cutaneous fragility induced by hypercorticism is an important cofactor permitting direct inoculation from the environment.

Intralesional therapy with anti-CD20 monoclonal antibody rituximab: local and systemic efficacy in primary cutaneous B-cell lymphoma.

Rituximab (MabThera®, Roche) is a chimeric monoclonal antibody directed against the CD20 antigen. Its efficacy and safety were first demonstrated in the treatment of systemic B‐cell lymphomas. We report the use of intralesional injections of rituximab into some but not all cutaneous lesions in a patient with multiple primary cutaneous follicular centre B‐cell lymphoma. This treatment resulted in tumour regression, even of the lesions that had not been injected. We therefore hypothesize that there is systemic diffusion of rituximab from injected sites despite the low doses injected locally, or the induction of a specific antitumour immune response acting systemically.

Oral pristinamycin versus standard penicillin regimen to treat erysipelas in adults: randomised, non-inferiority, open trial.

Abstract Objective: To assess the efficacy and safety of oral pristinamycin versus intravenous then oral penicillin to treat erysipelas in patients in hospital. Design: Multicentre, parallel group, open labelled, randomised non-inferiority trial. Setting: 22 French hospitals. Participants: 289 adults admitted to hospital with erysipelas. Results: At follow up (day 25-45) the cure rate (primary efficacy end point) for the per protocol populations was 81% (83/102) for pristinamycin and 67% (68/102) for penicillin. The planned interim analysis (global one sided type I error 5%) showed that the one sided 97.06% confidence interval of the observed difference (pristinamycin−penicillin) between cure rates (3.3% to ∞) exceeded the −10% non-inferiority threshold. For the intention to treat populations the cure rate at follow up was 65% (90/138) for pristinamycin and 53% (79/150) for penicillin, with the one sided 97.06% confidence interval of the observed difference between cure rates (1.7% to ∞) exceeding the −10% non-inferiority threshold. That the lower limit of the confidence interval exceeded the -10% threshold and was also >0 supports the hypothesis that pristinamycin is significantly superior at the 5% level. More adverse events related to treatment, as assessed by the investigators, were reported in the pristinamycin group than in the penicillin group. Most adverse events involved the gastrointestinal tract (nausea, vomiting, and diarrhoea) but were minor and usually did not require discontinuation of treatment. Conclusion: Pristinamycin could be an alternative to the standard intravenous then oral penicillin regimen used to treat erysipelas in adults in hospital, with the advantages of oral first line therapy.

Iododerma and acute respiratory distress with leucocytoclastic vasculitis following the intravenous injection of contrast medium

A 72‐year‐old woman with chronic renal failure requiring haemodialysis developed acute ioderma twice, after receiving iodide contrast dye for radiological procedures. Iododerma was localized to the face, scalp and elbows and was associated with papular purpura of the legs. Histopathology of the skin lesions showed acute necrortzing vasculitis. During the second skin eruption the patient developed acute respiratory distress, which was treated with corticosteroids. During the first eruption asymptomatic infiltrates were present on chest X‐ray which disappeared 2 months later. At the present time iododerma seems more frequent in patients with renal failure. Iodides may also be responsible for pulmonary abnormalities, which are sometimes asymptomatic. All these features may be due to leucocytoclastic vasculitis following iodide ingestion.

COEXISTENT CUTANEOUS T-CELL LYMPHOMA AND B-CELL MALIGNANCY

BACKGROUND The coexistence of cutaneous T-cell lymphoma (CTCL) and a B-cell malignancy (BCM) is rare. OBJECTIVE Our aim was to assess the clinical and pathologic aspects of coexistent CTCL and BCM and to examine potential explanations for this association. METHODS We report six cases of concurrent CTCL and BCM in which B- and T-cell lineages were demonstrated by immunologic studies. The literature includes 13 additional cases. All 19 CTCL-BCM cases are reviewed. RESULTS CTCL either preceded or followed the BCM, which was a low-grade malignancy in most cases (16 of 19). Possible explanations for the association include a genetic predisposition, underlying viral infection, chemotherapy-induced carcinogenesis, stimulation of a B-cell clone by malignant helper T cells, and alterations in progenitor cells before determination of B- and T-cell lineage. CONCLUSION An alteration in progenitor cells, with subsequent oncogenic activation of variable origin, might account for most cases of coexistent CTCL and BCM.