The Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI): grading disease severity and assessing responsiveness to clinical change in epidermolysis bullosa.

BACKGROUND: The lack of validated outcome measures for epidermolysis bullosa (EB) presents major barriers to evaluating disease severity and comparing the efficacy of therapies. The Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) was recently introduced as a valid and reliable instrument for EB; however, its interpretation for use in clinical practice and clinical trials is yet to be defined. OBJECTIVE: To assess the interpretability of the EBDASI in classifying patients according to disease severity and clinical response. METHODS: A total of 53 outpatients with EB at two interstate institutions were prospectively evaluated. At each visit, the principal dermatologist completed the EBDASI and global assessments of disease severity and change. Classifications for mild, moderate and severe disease using the EBDASI were determined using receiver operating characteristic curves. Minimal clinically important differences for the EBDASI activity subscale were calculated and compared with the standard error of measurement. RESULTS: Total EBDASI score ranges of 0-42, 43-106 and 107-506 corresponded to mild, moderate and severe disease respectively. Reduction in EBDASI activity scores of greater than 9 indicated clinically significant improvement. An increase of 3 in the activity score indicated deterioration. CONCLUSION: The EBDASI is a responsive tool and may be useful in characterizing disease severity and response. The cut-offs proposed in this study provide the first practical guide for interpreting the EBDASI, further supporting its use for longitudinal patient assessment and in clinical trials.

Threats to kill: a follow-up study.

BACKGROUND: Mental health clinicians are frequently asked to assess the risks presented by patients making threats to kill, but there are almost no data to guide such an evaluation. METHOD: This data linkage study examined serious violence following making threats to kill and the potential role of mental disorder. A total of 613 individuals convicted of threats to kill had their prior contact with public mental health services established at the time of the index offence. The group's subsequent criminal convictions were established 10 years later using the police database. Death from suicidal or homicidal violence was also established. RESULTS: Within 10 years, 44% of threateners were convicted of further violent offending, including 19 (3%) homicides. Those with histories of psychiatric contact (40%) had a higher rate (58%) of subsequent violence. The highest risks were in substance misusers, mentally disordered, young, and those without prior criminal convictions. Homicidal violence was most frequent among threateners with a schizophrenic illness. Sixteen threateners (2.6%) killed themselves, and three were murdered. CONCLUSIONS: In contrast to the claims in the literature that threats are not predictive of subsequent violence, this study revealed high rates of assault and even homicide following threats to kill. The mentally disordered were over-represented among threat offenders and among those at high risk of subsequent violence. The mentally disordered threateners at highest risk of violence were young, substance abusing, but not necessarily with prior convictions. Those who threaten others were also found to be at greater risk of killing themselves or being killed.

Comparison of 1- and 2-year screening intervals for women undergoing screening mammography.

We compared the long-term impact of 1- and 2-year screening mammography intervals using prognostic, screening, and outcome information for women aged 50-74 years obtained from the Screening Mammography Program of British Columbia in two time periods, prior to 1997 (policy of annual mammography) and after 1997 (biennial mammography). Survival was estimated for both periods using a prognostic model and the expected rate of interval and screen-detected cancers. The likelihood of a screen-detected cancer with annual screening was 2.32 per thousand screens and with biennial screening was 3.32 per thousand screens. The prognostic profile of screen-detected cancers was better than that of interval cancers. Among both screen-detected and interval cancers, the prognostic profiles with annual and biennial screening were similar. The estimated breast cancer-specific survival rates for women undergoing annual and biennial screening mammography were 95.2 and 94.6% at 5 years, and 90.4 and 89.2% at 10 years, respectively. Annual compared to biennial mammography was associated with a 1.2% increase in the estimated 10-year breast cancer-specific survival for women aged 50-74 years, diagnosed with invasive breast cancer after screening programme attendance.

Bullous pemphigoid induced by cephalexin.

Two young men developed severe bullous eruptions with a distinctive clinical picture of severe flexural involvement and extensive mucosal ulceration. Biopsies showed subepidermal bullae and associated inflammation consisted of predominantly neutrophils. Both had IgG and C3 staining of the dermal-epidermal junction on direct immunofluorescence. Bullous pemphigoid was diagnosed on the basis of clinical, histopathological and immunofluorescence findings. Both cases occurred after recent ingestion of cephalexin. We believe they represent the first reported cases of bullous pemphigoid induced by cephalexin.

Pseudomonas aeruginosa infection mimicking erythema annulare centrifugum.

A 3-year-old girl receiving chemotherapy for acute lymphocytic leukaemia developed a rapidly expanding red annular plaque on her thigh, initially without signs of systemic toxicity or local pain. Subsequently she developed Pseudomonas aeruginosa sepsis and purpura at the leading edge of the plaque. Skin biopsy showed an extensive necrotizing vasculitis with numerous Gram-negative bacilli in the blood vessel walls. In immunocompromised individuals, skin biopsy and culture of cutaneous lesions for bacteria and fungi should be considered even in the absence of signs of systemic toxicity or multiple lesions.

Avian mite dermatitis.

Avian mite infestation is a rare cause of pruritic dermatoses in humans. The mites spend the majority of their life cycle on the avian host but may be transmitted to man as a result of direct contact and also through airborne spread. We describe a case of infestation with the Northern Fowl Mite (Ornithonyssus sylviarum) from an abandoned birds nest in the roof of a patients home. This caused a pruritic erythematous papular eruption on exposed sites that settled with topical steroids. We discuss the diagnosis and range of clinical manifestations produced by avian mites.

DNA polymorphism analysis in transfusion-associated graft-versus-host disease.

During cardiac surgery for transposition of the great arteries at age 7 weeks, a female infant received blood, fresh frozen plasma and platelet transfusions. Eleven days postoperatively, she developed bloody diarrhoea, fever, an erythematous macular rash, hepatomegaly, seizures and pancytopaenia. A clinical diagnosis of transfusion related graft-versus-host disease (GVHD) was supported by skin histopathology. DNA polymorphism studies confirmed that circulating lymphocytes in peripheral blood and infiltrating cells in the skin were foreign in origin and were derived from transfused blood cells. No underlying immunodeficiency was identified. Treatment with steroids cyclosporin and antithymocyte globulin was unsuccessful and death occurred 2 months after surgery. The features of fever, rash, diarrhoea, liver dysfunction and pancytopaenia which characterize GVHD may mimic drug reactions or viral infection. In addition to histological features on skin biopsy. DNA polymorphism studies on skin and blood samples provide a unique and sensitive method to confirm GVHD. Irradiation of blood products should be considered for acutely compromised infants requiring urgent cardiac surgery.

Erythropoietic protoporphyria treated with narrow-band (TL-01) UVB phototherapy.

Erythropoietic protoporphyria is a rare photodermatosis for which treatment options are limited. The present report describes the clinical features of a patient with erythropoietic protoporphyria and liver function test abnormalities associated with treatment with beta-carotene. Subsequent treatment with narrow-band UVB phototherapy resulted in marked subjective improvement in photosensitivity, which was confirmed by abolition of demonstrated abnormalities on monochromator phototesting. The therapeutic options for photosensitivity in erythropoietic protoporphyria are reviewed and discussed.

Oesophageal involvement in cicatricial pemphigoid.

Four cases of cicatricial pemphigoid complicated by oesophageal involvement are presented. All patients suffered dysphagia but repeated radiological studies were required for confirmation of oesophageal ulceration, webs and strictures. A combination of systemic drug therapy and oesophageal dilatation were necessary for the suppression of symptoms. The clinical and immunopathological features, management and complications of oesophageal involvement in cicatricial pemphigoid are discussed. Dermatologists should be aware of these features and make regular inquiries about swallowing difficulties in patients with cicatricial pemphigoid to guide appropriate investigations and treatment.