Lehman, Julia S.

AbstractBackgroundMortality rates in patients with bullous pemphigoid (BP) are higher than those in age‐matched counterparts. However, the specific causes of death in BP subjects have not been evaluated systematically.ObjectivesWe sought to characterize the causes of death in patients with BP as recorded by death certificate and to compare these with death data for age‐ and location‐matched control subjects.MethodsThis was a retrospective cohort analysis conducted in a large tertiary referral center. Twenty‐seven participants who had a confirmed antemortem diagnosis of BP, were residents of Olmsted County, Minnesota, and had died between January 1, 1999, and January 1, 2009, were included in the study. Underlying cause of death and multiple causes of death data for the study population were compared with data sourced from the Centers for Disease Control and Prevention (CDC) for a control group matched by age and geographic location of origin by International Classification of Diseases, 10th Revision (ICD‐10) block, and specific ICD‐10 codes.ResultsComparisons of specific ICD‐10 codes revealed increased rates of sepsis (P = 0.031), dementia (P = 0.049), and major depressive disorder (P = 0.005) in the study group. The collective incidence of ICD‐10 codes for infections indicated that infections were more frequent contributors to death in the study group (P = 0.035).ConclusionsClinicians should be mindful of contributors to death in patients with BP and might consider screening for mental health issues, educating patients on the early symptoms of sepsis, and minimizing risk factors for infection.

Abstract Certain autoimmune bullous diseases ( AIBD ), including pemphigoid and pemphigus, confer increased infection risk. Infections have not been systematically studied in these conditions, however. Little is known about infection risk in these conditions, particularly dermatitis herpetiformis (DH). We aimed to characterize and compare infection patterns and risk factors in patients with pemphigoid, pemphigus, and DH. We retrospectively studied the medical records of Olmsted County, Minnesota, residents who had a diagnosis of AIBD between 1 January 1998 and 1 January 2011. Of 81 patients studied, 54 (67%) had pemphigoid, 11 (14%) had pemphigus and 16 (20%) had DH . Most patients studied developed at least one localized infection (72%) or one systemic infection (83%). Almost one‐third of patients (31%) developed infections requiring hospitalization or contributing to death. All patients taking systemic corticosteroids experienced a localized or systemic infection during the follow‐up period. Systemic infections were significantly less frequent in patients with DH than those with pemphigoid or pemphigus ( P = 0.03), as were systemic infections requiring hospitalization or contributing to death ( P = 0.002). Patients with DH were significantly less likely to require systemic corticosteroids ( P < 0.001) and significantly more likely to receive dapsone ( P = 0.002). The study design was retrospective and a limited number of patients met the inclusion criteria. Patients with AIBD frequently developed localized and systemic infections, a substantial portion of which contributed to hospitalization or death. Patients with DH experienced infections of lesser severity than patients with pemphigoid or pemphigus.

AbstractIntercellular adhesion proteins are poorly characterized in acantholytic squamous cell carcinoma (ASCC), a more aggressive tumor than nonacantholytic invasive well-differentiated squamous cell carcinoma (SCC) of the skin. In this study we compared expression of Dsg3, E-cadherin, and syndecan-1 in ASCC and SCC. Immunohistochemical detection of Dsg3, E-cadherin, and syndecan-1 in 22 ASCCs and 22 SCCs was graded on a semiquantitative scale for intensity of staining (SI) and degree of circumferential staining (CS) about the cell membrane. Results were assessed by means of conditional logistic regression and χ2 analysis. Dsg3 and E-cadherin expression (SI, CS) was significantly decreased (P &lt; .05) in ASCC compared with SCC, whereas staining for syndecan-1 was similar in the 2 tumor types. Differences in expression of adhesion markers between ASCC and SCC may contribute to the development of acantholysis in ASCC and its more aggressive biologic behavior.

Background Inherent to some immunobullous disorders is potential for intraepidermal or dermal–epidermal junction fragility, a phenomenon that may compromise biopsy specimen integrity and direct immunofluorescence (DIF) interpretation. In these situations, cutaneous adnexal structures (e.g. hair follicles, sweat apparatus) usually remain intact. Whether periadnexal DIF findings are reliable in diagnosing immunobullous conditions is unknown. Methods We evaluated 56 cutaneous specimens with diagnostic immunoglobulin (Ig) deposition patterns that contained adnexal structures. In a corollary study, we examined 145 hematoxylin‐eosin‐stained frozen specimens to determine biopsy factors associated with the presence of adnexal structures. Results Periadnexal DIF findings offered diagnostic sensitivity in conditions with linear or cell‐surface Ig deposition or lupus band. Periadnexal DIF findings were unreliable in dermatitis herpetiformis. Biopsy specimens from scalp and genitalia were most likely to contain folliculosebaceous units and sweat duct apparatus, respectively. Relative depth of biopsy correlated directly with the likelihood of identifying sweat duct apparatus but not folliculosebaceous units. Conclusions Periadnexal DIF findings may add diagnostic sensitivity in DIF evaluation of pemphigoid, pemphigus and lupus erythematosus. Pathologists can guide clinicians to biopsy certain anatomic sites and to obtain sufficient biopsy depth to increase the probability of capturing adnexal structures and, therefore, diagnostic yield from DIF specimens.