Hospitalization and mortality in Asian autoimmune bullous dermatosis patients: A 17-year retrospective study.

Limited data exist on the factors associated with hospitalization and mortality in Asian inpatients with autoimmune bullous dermatoses (AIBDs). This study aimed to elucidate the risk factors affecting hospitalization and mortality rates in Asian patients with AIBDs. A retrospective analysis of patients with AIBDs treated at Siriraj Hospital during a 17-year period was performed using the International Classification of Diseases 10th revision codes. The characteristics of inpatients and outpatients were compared, and mortality rates and associated factors were identified. The study included 360 AIBD patients (180 inpatients, 180 outpatients). Inpatients were significantly younger than outpatients. The identified risk factors for hospitalization were malignancy (odds ratio [OR] 2.83, 95% confidence interval [CI] 1.13-8.04; p = 0.034), moderate to severe disease (OR 2.52, 95% CI 1.49-4.34; p < 0.001), systemic corticosteroid use ≥15 mg/day (OR 2.27, 95% CI 1.21-4.41; p = 0.013) and oral cyclophosphamide treatment (OR 9.88, 95% CI 3.82-33.7; p < 0.001). Kaplan-Meier analysis revealed mortality rates of 26%, 36% and 39% for inpatients with pemphigus at 1, 3 and 5 years, respectively. For inpatients with pemphigoid, the corresponding rates were 28%, 38% and 47%. Infections, particularly pneumonia, were the predominant cause of death in both conditions. This study confirmed that both Asian ethnicity and healthcare disparities may be correlated with adverse outcomes in patients with AIBDs. Pemphigus mortality rates were substantially greater in Asian patients than in Caucasian patients. Continuous monitoring of factors contributing to hospitalization and mortality is imperative to improve treatment outcomes.

Mortality of bullous skin disorders from 1979 through 2002 in the United States.

OBJECTIVES: To identify and analyze trends in bullous disease mortality from 1979 through 2002 in the United States. DESIGN: Retrospective population-based analysis. SETTING: Mortality records from the Centers for Disease Control and Prevention mortality database. PARTICIPANTS: Mortality records from 1979 through 2002 for persons who died of bullous disease. MAIN OUTCOME MEASURES: Age-adjusted mortality rates and trends for 4 bullous disease subgroups: toxic epidermal necrolysis, pemphigoid, pemphigus, and epidermolysis bullosa. RESULTS: The overall age-adjusted (to the 2000 US standard population) annual mortality rate from bullous diseases of the skin was 0.103 death per 100 000. The average mortality from bullous disorders was 0.098 per 100 000 in 1979 through 1982 and remained stable at 0.099 per 100 000 during the final 4 years of the study, 1999 through 2002. Pemphigoid had a significant increase in mortality from 1979 through 2002, while pemphigus demonstrated a significant decrease in mortality. The mortality rate for toxic epidermal necrolysis was much higher among blacks (0.192 death per 100 000) than whites (0.025 per 100 000) (P < .001), with a mortality rate ratio of 7.57 (95% confidence interval, 6.97-8.21). CONCLUSIONS: Overall mortality from bullous diseases remained stable from 1979 through 2002, although an increasing mortality from pemphigoid and a decreasing mortality from pemphigus occurred during this period. A very large racial disparity in mortality from toxic epidermal necrolysis was observed.

[Severe cutaneous adverse drug reactions. Clinical features and epidemiology ]. / Schwere Arzneimittelreaktionen der Haut. Klinik und Epidemiologie.

Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are severe drug-induced bullous skin reactions. They are rare, but often life-threatening and have a high mortality rate. Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction, but after the suspected drug is withdrawn, the skin heals rapidly and mortality is low. The clinical pattern, histology and inducing drugs differ substantially between AGEP and the SJS/TEN group.

The fetal prognosis in pemphigoid gestationis (herpes gestationis).

We have studied the infants in fifty pregnancies affected by pemphigoid gestationis (herpes gestationis). There was a significant increase in the frequency of infants that were 'small for dates'. As such infants have a raised mortality and morbidity it follows that in pemphigoid gestationis the fetal prognosis is impaired. In view of this it is essential that patients with pemphigoid gestationis are delivered in maternity units which have facilities for intensive care of the newborn.

Some factors affecting prognosis in pemphigus vulgaris and pemphigoid.

The records of twenty-eight patients dying with pemphigus vulgaris, and forty-two with pemphigoid, have been examined for factors affecting the length of their survival. Age at onset of the disease is important, old patients dying significantly more quickly than young ones. A short pre-treatment phase signifies a fulminating course in pemphigus vulgaris but not in pemphigoid.

The events leading to the death of patients with pemphigus and pemphigoid.

This paper is based upon a study of all available records of patients certified as having died in hospital from pemphigus and pemphigoid in England and Wales from 1962 to 1969. The results differ from most published series in that many of the 210 patients died still with extensive skin lesions and with biochemical abnormalities, such as low serum albumin, sodium and chloride, which were secondary to this. Side-effects of treatment, such as diabetes, peptic ulceration, and infections, were also important but the commonest immediate causes of death were respiratory tract infections and pulmonary embolism.

International mortality from bullous diseases since 1950.

National mortality figures hold information about our overall success in treating bullous disorders which cannot be found elsewhere. The official data, since 1950, for England and Wales, the Unites States, France, Japan, Scotland, Ireland and Denmark have been analysed after corrections for changes in the population structure and in the International Classification of Disease. A steep fall in deaths occurred in England and Wales between 1952 (121 deaths) and 1955 (55 deaths). The low rate persisted from 1955 to 1962 (40 deaths) when it fell to a steady even lower level lasting until the present. The United States figures showed a sharp drop in mortality starting about 2 years earlier (from 283 deaths in 1950 to 150 deaths in 1954), followed by a gradual fall to the present. Mortality in Scotland, Ireland and Denmark has followed the same general pattern but the figures from France, after a steady fall between 1951 (68 deaths) and 1965 (29 deaths), have risen again until 1971 (62 deaths). The number of deaths has not fallen in Japan but the data are difficult to interpret. The timing of the improvement in mortality in England and Wales, and in the United States, fits well with the introduction of systemic steroids into general use. A fall to about one-third of the presteroid level is in general agreement with the improvement recorded in smaller personal series from several centres.