Kevan G. Lewis

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.

Mid‐dermal elastolysis preceded by acute neutrophilic dermatosis

Background:  Mid‐dermal elastolysis is a rare idiopathic elastic tissue disorder that is characterized by localized patches of finely wrinkled skin and a ‘band‐like’ loss of elastic tissue in the mid‐reticular dermis. Lesions may be preceded by erythema and/or urticaria, and histological examination of inflamed lesional skin may demonstrate lymphohistiocytic dermal infiltration.

Papillary dermal elastosis: a unique elastic tissue disorder or an unusual manifestation of pseudoxanthoma elasticum-like papillary dermal elastolysis?

There are numerous acquired elastic tissue disorders, several of which present cutaneously with small yellow‐to‐white papules resembling plucked chicken skin. Differential diagnoses depend on the abnormalities within the network of elastic tissues. We report a case with distinct histologic features, which may represent a unique elastic tissue disorder or a variant of pseudoxanthoma elasticum‐like papillary dermal elastolysis. Our patients clinical presentation includes scattered 1–2 mm white‐to‐yellow papules without surface change on the upper back and neck region. Histology is characterized by foci of clumped, granular elastic tissue, which have replaced the oxytalan and elaunin fibers, alternating with foci of decreased concentrations of normal‐appearing elastic fibers within the papillary dermis. Given its characteristics, we have termed this novel entity ‘papillary dermal elastosis’.

Superficial Flaccid Bullae in a Female Neonate—Quiz Case

A 58-year-old woman presented with Escherichia coli bacteremia, vancomycin-resistant Enterococcus faecium meningitis, and septic shock. Before this presentation, she had received systemic corticosteroids on 3 separate occasions at another hospital for exacerbations of chronic obstructive pulmonary disease as a result of 1-antitrypsin disease. Physical examination revealed an extensive purpuric rash on her abdomen (Figure1) and the anterior aspect of her thighs.Shediedshortlyafterward.Sectionsof skinobtained atautopsyshowedparasiticorganismsinthesuperficialdermis (Figure 2 and Figure 3) and subcutaneous fat. What is your diagnosis?